{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T22:47:35Z","timestamp":1782254855247,"version":"3.54.5"},"publisher-location":"Singapore","reference-count":321,"publisher":"Springer Singapore","isbn-type":[{"value":"9789811399121","type":"print"},{"value":"9789811399138","type":"electronic"}],"license":[{"start":{"date-parts":[[2019,1,1]],"date-time":"2019-01-01T00:00:00Z","timestamp":1546300800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019]]},"DOI":"10.1007\/978-981-13-9913-8_10","type":"book-chapter","created":{"date-parts":[[2019,10,3]],"date-time":"2019-10-03T13:04:46Z","timestamp":1570107886000},"page":"227-272","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Astrocytes in Motor Neuron Diseases"],"prefix":"10.1007","author":[{"given":"Chiara F.","family":"Valori","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Guidotti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liliana","family":"Brambilla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Rossi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2019,10,4]]},"reference":[{"key":"10_CR1","doi-asserted-by":"publisher","first-page":"9","DOI":"10.3109\/21678421.2013.805784","volume":"15","author":"S Abrahams","year":"2014","unstructured":"Abrahams S, Newton J, Niven E, Foley J, Bak TH (2014) Screening for cognition and behaviour changes in ALS. Amyotrophic lateral sclerosis and frontotemporal degeneration 15:9\u201314","journal-title":"Amyotrophic lateral sclerosis and frontotemporal degeneration"},{"key":"10_CR2","doi-asserted-by":"publisher","first-page":"754","DOI":"10.1038\/cdd.2010.143","volume":"18","author":"J Aebischer","year":"2011","unstructured":"Aebischer J, Cassina P, Otsmane B, Moumen A, Seilhean D, Meininger V, Barbeito L, Pettmann B, Raoul C (2011) IFNgamma triggers a LIGHT-dependent selective death of motoneurons contributing to the non-cell-autonomous effects of mutant SOD1. Cell Death Differ 18:754\u2013768","journal-title":"Cell Death Differ"},{"key":"10_CR3","doi-asserted-by":"publisher","first-page":"471","DOI":"10.1080\/21678421.2017.1369125","volume":"18","author":"A Al-Chalabi","year":"2017","unstructured":"Al-Chalabi A et al (2017) July 2017 ENCALS statement on edaravone. Amyotrophic lateral sclerosis and frontotemporal degeneration 18:471\u2013474","journal-title":"Amyotrophic lateral sclerosis and frontotemporal degeneration"},{"key":"10_CR4","doi-asserted-by":"publisher","first-page":"1252","DOI":"10.1002\/glia.22997","volume":"64","author":"S Al Awabdh","year":"2016","unstructured":"Al Awabdh S, Gupta-Agarwal S, Sheehan DF, Muir J, Norkett R, Twelvetrees AE, Griffin LD, Kittler JT (2016) Neuronal activity mediated regulation of glutamate transporter GLT-1 surface diffusion in rat astrocytes in dissociated and slice cultures. Glia 64:1252\u20131264","journal-title":"Glia"},{"key":"10_CR5","doi-asserted-by":"publisher","first-page":"1154","DOI":"10.1002\/glia.22989","volume":"64","author":"AA Almad","year":"2016","unstructured":"Almad AA, Doreswamy A, Gross SK, Richard JP, Huo Y, Haughey N, Maragakis NJ (2016) Connexin 43 in astrocytes contributes to motor neuron toxicity in amyotrophic lateral sclerosis. Glia 64:1154\u20131169","journal-title":"Glia"},{"key":"10_CR6","doi-asserted-by":"publisher","first-page":"2554","DOI":"10.1212\/WNL.0000000000003427","volume":"87","author":"MJ Alshikho","year":"2016","unstructured":"Alshikho MJ, Zurcher NR, Loggia ML, Cernasov P, Chonde DB, Izquierdo Garcia D, Yasek JE, Akeju O, Catana C, Rosen BR, Cudkowicz ME, Hooker JM, Atassi N (2016) Glial activation colocalizes with structural abnormalities in amyotrophic lateral sclerosis. Neurology 87:2554\u20132561","journal-title":"Neurology"},{"key":"10_CR7","doi-asserted-by":"crossref","unstructured":"Alshikho MJ, Zurcher NR, Loggia ML, Cernasov P, Reynolds B, Pijanowski O, Chonde DB, Izquierdo Garcia D, Mainero C, Catana C, Chan J, Babu S, Paganoni S, Hooker JM, Atassi N (2018) Integrated MRI and [(11) C]-PBR28 PET Imaging in Amyotrophic Lateral sclerosis. Ann Neurol","DOI":"10.1212\/WNL.88.16_supplement.P3.139"},{"key":"10_CR8","doi-asserted-by":"publisher","first-page":"418","DOI":"10.1006\/exnr.2001.7756","volume":"171","author":"JM Anneser","year":"2001","unstructured":"Anneser JM, Cookson MR, Ince PG, Shaw PJ, Borasio GD (2001) Glial cells of the spinal cord and subcortical white matter up-regulate neuronal nitric oxide synthase in sporadic amyotrophic lateral sclerosis. Exp Neurol 171:418\u2013421","journal-title":"Exp Neurol"},{"key":"10_CR9","doi-asserted-by":"publisher","first-page":"645","DOI":"10.1002\/ana.410430514","volume":"43","author":"M Aoki","year":"1998","unstructured":"Aoki M, Lin CL, Rothstein JD, Geller BA, Hosler BA, Munsat TL, Horvitz HR, Brown RH Jr (1998) Mutations in the glutamate transporter EAAT2 gene do not cause abnormal EAAT2 transcripts in amyotrophic lateral sclerosis. Ann Neurol 43:645\u2013653","journal-title":"Ann Neurol"},{"key":"10_CR10","doi-asserted-by":"publisher","first-page":"441","DOI":"10.1007\/s00401-003-0743-9","volume":"106","author":"S Araki","year":"2003","unstructured":"Araki S, Hayashi M, Tamagawa K, Saito M, Kato S, Komori T, Sakakihara Y, Mizutani T, Oda M (2003) Neuropathological analysis in spinal muscular atrophy type II. Acta Neuropathol 106:441\u2013448","journal-title":"Acta Neuropathol"},{"key":"10_CR11","doi-asserted-by":"publisher","first-page":"16060","DOI":"10.1038\/mtm.2016.60","volume":"3","author":"N Armbruster","year":"2016","unstructured":"Armbruster N, Lattanzi A, Jeavons M, Van Wittenberghe L, Gjata B, Marais T, Martin S, Vignaud A, Voit T, Mavilio F, Barkats M, Buj-Bello A (2016) Efficacy and biodistribution analysis of intracerebroventricular administration of an optimized scAAV9-SMN1 vector in a mouse model of spinal muscular atrophy. Mol Ther Methods Clin Dev 3:16060","journal-title":"Mol Ther Methods Clin Dev"},{"key":"10_CR12","doi-asserted-by":"publisher","first-page":"509","DOI":"10.1016\/S0306-4522(01)00181-6","volume":"105","author":"E Aronica","year":"2001","unstructured":"Aronica E, Catania MV, Geurts J, Yankaya B, Troost D (2001) Immunohistochemical localization of group I and II metabotropic glutamate receptors in control and amyotrophic lateral sclerosis human spinal cord: upregulation in reactive astrocytes. Neuroscience 105:509\u2013520","journal-title":"Neuroscience"},{"key":"10_CR13","doi-asserted-by":"publisher","first-page":"1726","DOI":"10.1172\/JCI22922","volume":"114","author":"M Azzouz","year":"2004","unstructured":"Azzouz M, Le T, Ralph GS, Walmsley L, Monani UR, Lee DC, Wilkes F, Mitrophanous KA, Kingsman SM, Burghes AH, Mazarakis ND (2004) Lentivector-mediated SMN replacement in a mouse model of spinal muscular atrophy. J Clin Investig 114:1726\u20131731","journal-title":"J Clin Investig"},{"key":"10_CR14","first-page":"410","volume":"9","author":"DJ Baker","year":"2015","unstructured":"Baker DJ, Blackburn DJ, Keatinge M, Sokhi D, Viskaitis P, Heath PR, Ferraiuolo L, Kirby J, Shaw PJ (2015) Lysosomal and phagocytic activity is increased in astrocytes during disease progression in the SOD1 (G93A) mouse model of amyotrophic lateral sclerosis. Front Cell Neurosci 9:410","journal-title":"Front Cell Neurosci"},{"key":"10_CR15","doi-asserted-by":"publisher","first-page":"2385","DOI":"10.1128\/MCB.00087-15","volume":"35","author":"N Bakkar","year":"2015","unstructured":"Bakkar N, Kousari A, Kovalik T, Li Y, Bowser R (2015) RBM45 Modulates the Antioxidant Response in Amyotrophic Lateral Sclerosis through Interactions with KEAP1. Mol Cell Biol 35:2385\u20132399","journal-title":"Mol Cell Biol"},{"key":"10_CR16","doi-asserted-by":"publisher","first-page":"1991","DOI":"10.1002\/glia.22414","volume":"60","author":"D Bataveljic","year":"2012","unstructured":"Bataveljic D, Nikolic L, Milosevic M, Todorovic N, Andjus PR (2012) Changes in the astrocytic aquaporin-4 and inwardly rectifying potassium channel expression in the brain of the amyotrophic lateral sclerosis SOD1(G93A) rat model. Glia 60:1991\u20132003","journal-title":"Glia"},{"key":"10_CR17","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1038\/nrn.2016.159","volume":"18","author":"L Ben Haim","year":"2017","unstructured":"Ben Haim L, Rowitch DH (2017) Functional diversity of astrocytes in neural circuit regulation. Nat Rev Neurosci 18:31\u201341","journal-title":"Nat Rev Neurosci"},{"key":"10_CR18","doi-asserted-by":"publisher","first-page":"35899","DOI":"10.1074\/jbc.M112.366419","volume":"287","author":"V Benedusi","year":"2012","unstructured":"Benedusi V, Martorana F, Brambilla L, Maggi A, Rossi D (2012) The peroxisome proliferator-activated receptor gamma (PPARgamma) controls natural protective mechanisms against lipid peroxidation in amyotrophic lateral sclerosis. J Biol Chem 287:35899\u201335911","journal-title":"J Biol Chem"},{"key":"10_CR19","doi-asserted-by":"publisher","first-page":"282","DOI":"10.1038\/mt.2012.261","volume":"21","author":"S Benkhelifa-Ziyyat","year":"2013","unstructured":"Benkhelifa-Ziyyat S, Besse A, Roda M, Duque S, Astord S, Carcenac R, Marais T, Barkats M (2013) Intramuscular scAAV9-SMN injection mediates widespread gene delivery to the spinal cord and decreases disease severity in SMA mice. Mol Ther: J Am Soc Gene Ther 21:282\u2013290","journal-title":"Mol Ther: J Am Soc Gene Ther"},{"key":"10_CR20","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1007\/s12031-015-0695-2","volume":"58","author":"C Benkler","year":"2016","unstructured":"Benkler C, Barhum Y, Ben-Zur T, Offen D (2016) Multifactorial gene therapy enhancing the glutamate uptake system and reducing oxidative stress delays symptom onset and prolongs survival in the SOD1-G93A ALS mouse model. J Mol Neurosci: MN 58:46\u201358","journal-title":"J Mol Neurosci: MN"},{"key":"10_CR21","doi-asserted-by":"publisher","first-page":"130","DOI":"10.3109\/21678421.2013.839708","volume":"15","author":"P Bergstrom","year":"2014","unstructured":"Bergstrom P, von Otter M, Nilsson S, Nilsson AC, Nilsson M, Andersen PM, Hammarsten O, Zetterberg H (2014) Association of NFE2L2 and KEAP1 haplotypes with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Front Degener 15:130\u2013137","journal-title":"Amyotroph Lateral Scler Front Degener"},{"key":"10_CR22","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1016\/j.jneuroim.2013.08.014","volume":"264","author":"D Bernardes","year":"2013","unstructured":"Bernardes D, Oliveira-Lima OC, Silva TV, Faraco CC, Leite HR, Juliano MA, Santos DM, Bethea JR, Brambilla R, Orian JM, Arantes RM, Carvalho-Tavares J (2013) Differential brain and spinal cord cytokine and BDNF levels in experimental autoimmune encephalomyelitis are modulated by prior and regular exercise. J Neuroimmunol 264:24\u201334","journal-title":"J Neuroimmunol"},{"key":"10_CR23","doi-asserted-by":"publisher","first-page":"e61177","DOI":"10.1371\/journal.pone.0061177","volume":"8","author":"JD Berry","year":"2013","unstructured":"Berry JD, Shefner JM, Conwit R, Schoenfeld D, Keroack M, Felsenstein D, Krivickas L, David WS, Vriesendorp F, Pestronk A, Caress JB, Katz J, Simpson E, Rosenfeld J, Pascuzzi R, Glass J, Rezania K, Rothstein JD, Greenblatt DJ, Cudkowicz ME (2013) Design and initial results of a multi-phase randomized trial of ceftriaxone in amyotrophic lateral sclerosis. PLoS ONE 8:e61177","journal-title":"PLoS ONE"},{"key":"10_CR24","doi-asserted-by":"publisher","first-page":"4069","DOI":"10.1073\/pnas.1218497110","volume":"110","author":"F Bi","year":"2013","unstructured":"Bi F, Huang C, Tong J, Qiu G, Huang B, Wu Q, Li F, Xu Z, Bowser R, Xia XG, Zhou H (2013) Reactive astrocytes secrete lcn2 to promote neuron death. Proc Natl Acad Sci USA 110:4069\u20134074","journal-title":"Proc Natl Acad Sci USA"},{"key":"10_CR25","doi-asserted-by":"publisher","first-page":"1271","DOI":"10.1111\/j.1471-4159.2008.05699.x","volume":"107","author":"LG Bilsland","year":"2008","unstructured":"Bilsland LG, Nirmalananthan N, Yip J, Greensmith L, Duchen MR (2008) Expression of mutant SOD1 in astrocytes induces functional deficits in motoneuron mitochondria. J Neurochem 107:1271\u20131283","journal-title":"J Neurochem"},{"key":"10_CR26","doi-asserted-by":"publisher","first-page":"21","DOI":"10.1016\/0022-510X(96)00110-4","volume":"140","author":"HG Blaauwgeers","year":"1996","unstructured":"Blaauwgeers HG, Vianney de Jong JM, Verspaget HW, van den Berg FM, Troost D (1996) Enhanced superoxide dismutase-2 immunoreactivity of astrocytes and occasional neurons in amyotrophic lateral sclerosis. J Neurol Sci 140:21\u201329","journal-title":"J Neurol Sci"},{"key":"10_CR27","doi-asserted-by":"publisher","first-page":"6","DOI":"10.3389\/fnana.2016.00006","volume":"10","author":"M Boido","year":"2016","unstructured":"Boido M, Vercelli A (2016) Neuromuscular Junctions as key contributors and therapeutic targets in spinal muscular atrophy. Front Neuroanat 10:6","journal-title":"Front Neuroanat"},{"key":"10_CR28","doi-asserted-by":"publisher","first-page":"14076","DOI":"10.1074\/jbc.M600653200","volume":"281","author":"W Boston-Howes","year":"2006","unstructured":"Boston-Howes W, Gibb SL, Williams EO, Pasinelli P, Brown RH Jr, Trotti D (2006) Caspase-3 cleaves and inactivates the glutamate transporter EAAT2. J Biol Chem 281:14076\u201314084","journal-title":"J Biol Chem"},{"key":"10_CR29","doi-asserted-by":"crossref","unstructured":"Brambilla L, Martorana F, Guidotti G, Rossi D (2018) Dysregulation of astrocytic HMGB1 signaling in amyotrophic lateral sclerosis. Front Neurosci 12","DOI":"10.3389\/fnins.2018.00622"},{"key":"10_CR30","first-page":"3080","volume":"25","author":"L Brambilla","year":"2016","unstructured":"Brambilla L, Guidotti G, Martorana F, Iyer AM, Aronica E, Valori CF, Rossi D (2016) Disruption of the astrocytic TNFR1-GDNF axis accelerates motor neuron degeneration and disease progression in amyotrophic lateral sclerosis. Hum Mol Genet 25:3080\u20133095","journal-title":"Hum Mol Genet"},{"key":"10_CR31","doi-asserted-by":"publisher","first-page":"327","DOI":"10.1016\/S0896-6273(00)80272-X","volume":"18","author":"LI Bruijn","year":"1997","unstructured":"Bruijn LI, Becher MW, Lee MK, Anderson KL, Jenkins NA, Copeland NG, Sisodia SS, Rothstein JD, Borchelt DR, Price DL, Cleveland DW (1997) ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions. Neuron 18:327\u2013338","journal-title":"Neuron"},{"key":"10_CR32","doi-asserted-by":"publisher","first-page":"228","DOI":"10.1016\/j.neuroscience.2018.03.022","volume":"379","author":"D Buonvicino","year":"2018","unstructured":"Buonvicino D, Felici R, Ranieri G, Caramelli R, Lapucci A, Cavone L, Muzzi M, Di Pietro L, Bernardini C, Zwergel C, Valente S, Mai A, Chiarugi A (2018) Effects of class II-selective histone deacetylase inhibitor on neuromuscular function and disease progression in SOD1-ALS Mice. Neuroscience 379:228\u2013238","journal-title":"Neuroscience"},{"key":"10_CR33","doi-asserted-by":"publisher","first-page":"11424","DOI":"10.3390\/ijms140611424","volume":"14","author":"V Caraballo-Miralles","year":"2013","unstructured":"Caraballo-Miralles V, Cardona-Rossinyol A, Garcera A, Torres-Benito L, Soler RM, Tabares L, Llado J, Olmos G (2013) Notch signaling pathway is activated in motoneurons of spinal muscular atrophy. Int J Mol Sci 14:11424\u201311437","journal-title":"Int J Mol Sci"},{"key":"10_CR34","doi-asserted-by":"publisher","first-page":"38","DOI":"10.1111\/j.1471-4159.2004.02984.x","volume":"93","author":"P Cassina","year":"2005","unstructured":"Cassina P, Pehar M, Vargas MR, Castellanos R, Barbeito AG, Estevez AG, Thompson JA, Beckman JS, Barbeito L (2005) Astrocyte activation by fibroblast growth factor-1 and motor neuron apoptosis: implications for amyotrophic lateral sclerosis. J Neurochem 93:38\u201346","journal-title":"J Neurochem"},{"key":"10_CR35","doi-asserted-by":"publisher","first-page":"4115","DOI":"10.1523\/JNEUROSCI.5308-07.2008","volume":"28","author":"P Cassina","year":"2008","unstructured":"Cassina P, Cassina A, Pehar M, Castellanos R, Gandelman M, de Leon A, Robinson KM, Mason RP, Beckman JS, Barbeito L, Radi R (2008) Mitochondrial dysfunction in SOD1G93A-bearing astrocytes promotes motor neuron degeneration: prevention by mitochondrial-targeted antioxidants. J Neurosci: Off J Soc Neurosci 28:4115\u20134122","journal-title":"J Neurosci: Off J Soc Neurosci"},{"key":"10_CR36","doi-asserted-by":"publisher","first-page":"103","DOI":"10.1016\/j.neulet.2016.12.049","volume":"639","author":"GN Chan","year":"2017","unstructured":"Chan GN, Evans RA, Banks DB, Mesev EV, Miller DS, Cannon RE (2017) Selective induction of P-glycoprotein at the CNS barriers during symptomatic stage of an ALS animal model. Neurosci Lett 639:103\u2013113","journal-title":"Neurosci Lett"},{"key":"10_CR37","first-page":"646","volume":"25","author":"H Chen","year":"2018","unstructured":"Chen H, Kankel MW, Su SC, Han SWS, Ofengeim D (2018) Exploring the genetics and non-cell autonomous mechanisms underlying ALS\/FTLD. Cell Death Differ 25:646\u2013660","journal-title":"Cell Death Differ"},{"key":"10_CR38","doi-asserted-by":"crossref","unstructured":"Chen H, Qian K, Chen W, Hu B, Blackbourn LWt, Du Z, Ma L, Liu H, Knobel KM, Ayala M, Zhang SC (2015) Human-derived neural progenitors functionally replace astrocytes in adult mice. J Clin Investig 125:1033\u20131042","DOI":"10.1172\/JCI69097"},{"key":"10_CR39","doi-asserted-by":"publisher","first-page":"397","DOI":"10.1016\/j.bbrc.2012.03.006","volume":"420","author":"Y Chen","year":"2012","unstructured":"Chen Y, Guan Y, Liu H, Wu X, Yu L, Wang S, Zhao C, Du H, Wang X (2012) Activation of the Wnt\/beta-catenin signaling pathway is associated with glial proliferation in the adult spinal cord of ALS transgenic mice. Biochem Biophys Res Commun 420:397\u2013403","journal-title":"Biochem Biophys Res Commun"},{"key":"10_CR40","doi-asserted-by":"publisher","first-page":"890","DOI":"10.1212\/WNL.0000000000002445","volume":"86","author":"CA Chiriboga","year":"2016","unstructured":"Chiriboga CA, Swoboda KJ, Darras BT, Iannaccone ST, Montes J, De Vivo DC, Norris DA, Bennett CF, Bishop KM (2016) Results from a phase 1 study of nusinersen (ISIS-SMN(Rx)) in children with spinal muscular atrophy. Neurology 86:890\u2013897","journal-title":"Neurology"},{"key":"10_CR41","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1126\/science.1086071","volume":"302","author":"AM Clement","year":"2003","unstructured":"Clement AM, Nguyen MD, Roberts EA, Garcia ML, Boillee S, Rule M, McMahon AP, Doucette W, Siwek D, Ferrante RJ, Brown RH Jr, Julien JP, Goldstein LS, Cleveland DW (2003) Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice. Science 302:113\u2013117","journal-title":"Science"},{"key":"10_CR42","doi-asserted-by":"publisher","first-page":"653","DOI":"10.1177\/1087057110370998","volume":"15","author":"CK Colton","year":"2010","unstructured":"Colton CK, Kong Q, Lai L, Zhu MX, Seyb KI, Cuny GD, Xian J, Glicksman MA, Lin CL (2010) Identification of translational activators of glial glutamate transporter EAAT2 through cell-based high-throughput screening: an approach to prevent excitotoxicity. J Biomol Screen 15:653\u2013662","journal-title":"J Biomol Screen"},{"key":"10_CR43","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1007\/s00401-015-1429-9","volume":"130","author":"J Cooper-Knock","year":"2015","unstructured":"Cooper-Knock J, Higginbottom A, Stopford MJ, Highley JR, Ince PG, Wharton SB, Pickering-Brown S, Kirby J, Hautbergue GM, Shaw PJ (2015) Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy. Acta Neuropathol 130:63\u201375","journal-title":"Acta Neuropathol"},{"key":"10_CR44","doi-asserted-by":"publisher","first-page":"e17187","DOI":"10.1371\/journal.pone.0017187","volume":"6","author":"C Crosio","year":"2011","unstructured":"Crosio C, Valle C, Casciati A, Iaccarino C, Carri MT (2011) Astroglial inhibition of NF-kappaB does not ameliorate disease onset and progression in a mouse model for amyotrophic lateral sclerosis (ALS). PLoS ONE 6:e17187","journal-title":"PLoS ONE"},{"key":"10_CR45","doi-asserted-by":"publisher","first-page":"1083","DOI":"10.1016\/S1474-4422(14)70222-4","volume":"13","author":"ME Cudkowicz","year":"2014","unstructured":"Cudkowicz ME et al (2014) Safety and efficacy of ceftriaxone for amyotrophic lateral sclerosis: a multi-stage, randomised, double-blind, placebo-controlled trial. Lancet Neurol 13:1083\u20131091","journal-title":"Lancet Neurol"},{"key":"10_CR46","doi-asserted-by":"publisher","first-page":"1739","DOI":"10.3892\/mmr.2017.6786","volume":"16","author":"J Dai","year":"2017","unstructured":"Dai J, Lin W, Zheng M, Liu Q, He B, Luo C, Lu X, Pei Z, Su H, Yao X (2017) Alterations in AQP4 expression and polarization in the course of motor neuron degeneration in SOD1G93A mice. Mol Med Rep 16:1739\u20131746","journal-title":"Mol Med Rep"},{"key":"10_CR47","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1016\/j.neuron.2011.09.011","volume":"72","author":"M DeJesus-Hernandez","year":"2011","unstructured":"DeJesus-Hernandez M et al (2011) Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Neuron 72:245\u2013256","journal-title":"Neuron"},{"key":"10_CR48","doi-asserted-by":"publisher","first-page":"211","DOI":"10.1038\/nature10353","volume":"477","author":"HX Deng","year":"2011","unstructured":"Deng HX et al (2011) Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS\/dementia. Nature 477:211\u2013215","journal-title":"Nature"},{"key":"10_CR49","doi-asserted-by":"publisher","first-page":"637","DOI":"10.1016\/j.stem.2008.09.017","volume":"3","author":"FP Giorgio Di","year":"2008","unstructured":"Di Giorgio FP, Boulting GL, Bobrowicz S, Eggan KC (2008) Human embryonic stem cell-derived motor neurons are sensitive to the toxic effect of glial cells carrying an ALS-causing mutation. Cell Stem Cell 3:637\u2013648","journal-title":"Cell Stem Cell"},{"key":"10_CR50","doi-asserted-by":"publisher","first-page":"608","DOI":"10.1038\/nn1885","volume":"10","author":"FP Giorgio Di","year":"2007","unstructured":"Di Giorgio FP, Carrasco MA, Siao MC, Maniatis T, Eggan K (2007) Non-cell autonomous effect of glia on motor neurons in an embryonic stem cell-based ALS model. Nat Neurosci 10:608\u2013614","journal-title":"Nat Neurosci"},{"key":"10_CR51","doi-asserted-by":"crossref","unstructured":"Diaper DC, Adachi Y, Lazarou L, Greenstein M, Simoes FA, Di Domenico A, Solomon DA, Lowe S, Alsubaie R, Cheng D, Buckley S, Humphrey DM, Shaw CE, Hirth F (2013) Drosophila TDP-43 dysfunction in glia and muscle cells cause cytological and behavioural phenotypes that characterize ALS and FTLD. Hum Mol Genet","DOI":"10.1093\/hmg\/ddt243"},{"key":"10_CR52","doi-asserted-by":"publisher","first-page":"18126","DOI":"10.1073\/pnas.1110689108","volume":"108","author":"P Diaz-Amarilla","year":"2011","unstructured":"Diaz-Amarilla P, Olivera-Bravo S, Trias E, Cragnolini A, Martinez-Palma L, Cassina P, Beckman J, Barbeito L (2011) Phenotypically aberrant astrocytes that promote motoneuron damage in a model of inherited amyotrophic lateral sclerosis. Proc Natl Acad Sci USA 108:18126\u201318131","journal-title":"Proc Natl Acad Sci USA"},{"key":"10_CR53","doi-asserted-by":"publisher","first-page":"112","DOI":"10.1016\/j.freeradbiomed.2016.03.013","volume":"95","author":"P Diaz-Amarilla","year":"2016","unstructured":"Diaz-Amarilla P, Miquel E, Trostchansky A, Trias E, Ferreira AM, Freeman BA, Cassina P, Barbeito L, Vargas MR, Rubbo H (2016) Electrophilic nitro-fatty acids prevent astrocyte-mediated toxicity to motor neurons in a cell model of familial amyotrophic lateral sclerosis via nuclear factor erythroid 2-related factor activation. Free Radic Biol Med 95:112\u2013120","journal-title":"Free Radic Biol Med"},{"key":"10_CR54","doi-asserted-by":"publisher","first-page":"391","DOI":"10.1016\/j.nmd.2013.02.001","volume":"23","author":"N Dlamini","year":"2013","unstructured":"Dlamini N, Josifova DJ, Paine SM, Wraige E, Pitt M, Murphy AJ, King A, Buk S, Smith F, Abbs S, Sewry C, Jacques TS, Jungbluth H (2013) Clinical and neuropathological features of X-linked spinal muscular atrophy (SMAX2) associated with a novel mutation in the UBA1 gene. Neuromuscul Disord: NMD 23:391\u2013398","journal-title":"Neuromuscul Disord: NMD"},{"key":"10_CR55","doi-asserted-by":"publisher","first-page":"681","DOI":"10.1093\/hmg\/ddq514","volume":"20","author":"E Dominguez","year":"2011","unstructured":"Dominguez E, Marais T, Chatauret N, Benkhelifa-Ziyyat S, Duque S, Ravassard P, Carcenac R, Astord S, Pereira de Moura A, Voit T, Barkats M (2011) Intravenous scAAV9 delivery of a codon-optimized SMN1 sequence rescues SMA mice. Hum Mol Genet 20:681\u2013693","journal-title":"Hum Mol Genet"},{"key":"10_CR56","doi-asserted-by":"publisher","first-page":"1621","DOI":"10.1016\/j.neuroscience.2010.06.018","volume":"169","author":"W Duan","year":"2010","unstructured":"Duan W, Li X, Shi J, Guo Y, Li Z, Li C (2010) Mutant TAR DNA-binding protein-43 induces oxidative injury in motor neuron-like cell. Neuroscience 169:1621\u20131629","journal-title":"Neuroscience"},{"key":"10_CR57","doi-asserted-by":"publisher","first-page":"399","DOI":"10.1002\/ana.24332","volume":"77","author":"SI Duque","year":"2015","unstructured":"Duque SI, Arnold WD, Odermatt P, Li X, Porensky PN, Schmelzer L, Meyer K, Kolb SJ, Schumperli D, Kaspar BK, Burghes AH (2015) A large animal model of spinal muscular atrophy and correction of phenotype. Ann Neurol 77:399\u2013414","journal-title":"Ann Neurol"},{"key":"10_CR58","doi-asserted-by":"publisher","first-page":"15","DOI":"10.3389\/fncel.2018.00015","volume":"12","author":"N Duval","year":"2018","unstructured":"Duval N, Sumner WA, Andrianakos AG, Gray JJ, Bouchard RJ, Wilkins HM, Linseman DA (2018) The Bcl-2 Homology-3 Domain (BH3)-only proteins, bid, DP5\/Hrk, and BNip3L, are upregulated in reactive astrocytes of end-stage mutant SOD1 mouse spinal cord. Front Cell Neurosci 12:15","journal-title":"Front Cell Neurosci"},{"key":"10_CR59","doi-asserted-by":"publisher","first-page":"277","DOI":"10.1038\/nature07677","volume":"457","author":"AD Ebert","year":"2009","unstructured":"Ebert AD, Yu J, Rose FF Jr, Mattis VB, Lorson CL, Thomson JA, Svendsen CN (2009) Induced pluripotent stem cells from a spinal muscular atrophy patient. Nature 457:277\u2013280","journal-title":"Nature"},{"key":"10_CR60","first-page":"685","volume":"1852","author":"BM Edens","year":"2015","unstructured":"Edens BM, Ajroud-Driss S, Ma L, Ma YC (2015) Molecular mechanisms and animal models of spinal muscular atrophy. Biochem Biophys Acta 1852:685\u2013692","journal-title":"Biochem Biophys Acta"},{"key":"10_CR61","doi-asserted-by":"crossref","unstructured":"Edens BM, Yan J, Miller N, Deng HX, Siddique T, Ma YC (2017) A novel ALS-associated variant in UBQLN4 regulates motor axon morphogenesis. eLife 6","DOI":"10.7554\/eLife.25453"},{"key":"10_CR62","doi-asserted-by":"publisher","first-page":"687","DOI":"10.1111\/j.1751-7176.2011.00513.x","volume":"13","author":"WJ Elliott","year":"2011","unstructured":"Elliott WJ, Ram CV (2011) Calcium channel blockers. J Clin Hypertens (Greenwich) 13:687\u2013689","journal-title":"Calcium channel blockers. J Clin Hypertens (Greenwich)"},{"key":"10_CR63","doi-asserted-by":"publisher","first-page":"592","DOI":"10.1016\/j.celrep.2015.03.053","volume":"11","author":"F Endo","year":"2015","unstructured":"Endo F, Komine O, Fujimori-Tonou N, Katsuno M, Jin S, Watanabe S, Sobue G, Dezawa M, Wyss-Coray T, Yamanaka K (2015) Astrocyte-derived TGF-beta1 accelerates disease progression in ALS mice by interfering with the neuroprotective functions of microglia and T cells. Cell Rep 11:592\u2013604","journal-title":"Cell Rep"},{"key":"10_CR64","doi-asserted-by":"crossref","unstructured":"Estes PS, Daniel SG, McCallum AP, Boehringer AV, Sukhina AS, Zwick RA, Zarnescu DC (2013) Motor neurons and glia exhibit specific, individualized responses to TDP-43 expression in a Drosophila model of ALS. Dis Model Mech","DOI":"10.1242\/dmm.010710"},{"key":"10_CR65","doi-asserted-by":"publisher","first-page":"416","DOI":"10.1016\/S1474-4422(18)30054-1","volume":"17","author":"T Fang","year":"2018","unstructured":"Fang T, Al Khleifat A, Meurgey JH, Jones A, Leigh PN, Bensimon G, Al-Chalabi A (2018) Stage at which riluzole treatment prolongs survival in patients with amyotrophic lateral sclerosis: a retrospective analysis of data from a dose-ranging study. Lancet Neurol 17:416\u2013422","journal-title":"Lancet Neurol"},{"key":"10_CR66","doi-asserted-by":"publisher","first-page":"300","DOI":"10.3389\/fncel.2017.00300","volume":"11","author":"WT Farmer","year":"2017","unstructured":"Farmer WT, Murai K (2017) Resolving Astrocyte Heterogeneity in the CNS. Frontiers in cellular neuroscience 11:300","journal-title":"Frontiers in cellular neuroscience"},{"key":"10_CR67","doi-asserted-by":"publisher","first-page":"1440","DOI":"10.1001\/archneurol.2011.250","volume":"68","author":"F Fecto","year":"2011","unstructured":"Fecto F, Yan J, Vemula SP, Liu E, Yang Y, Chen W, Zheng JG, Shi Y, Siddique N, Arrat H, Donkervoort S, Ajroud-Driss S, Sufit RL, Heller SL, Deng HX, Siddique T (2011) SQSTM1 mutations in familial and sporadic amyotrophic lateral sclerosis. Arch Neurol 68:1440\u20131446","journal-title":"Arch Neurol"},{"key":"10_CR68","doi-asserted-by":"publisher","first-page":"1004","DOI":"10.1016\/j.neuropharm.2011.10.009","volume":"62","author":"X Feng","year":"2012","unstructured":"Feng X, Peng Y, Liu M, Cui L (2012) DL-3-n-butylphthalide extends survival by attenuating glial activation in a mouse model of amyotrophic lateral sclerosis. Neuropharmacology 62:1004\u20131010","journal-title":"Neuropharmacology"},{"key":"10_CR69","doi-asserted-by":"publisher","first-page":"2627","DOI":"10.1093\/brain\/awr193","volume":"134","author":"L Ferraiuolo","year":"2011","unstructured":"Ferraiuolo L, Higginbottom A, Heath PR, Barber S, Greenald D, Kirby J, Shaw PJ (2011) Dysregulation of astrocyte-motoneuron cross-talk in mutant superoxide dismutase 1-related amyotrophic lateral sclerosis. Brain: J Neurol 134:2627\u20132641","journal-title":"Brain: J Neurol"},{"key":"10_CR70","doi-asserted-by":"publisher","first-page":"3017","DOI":"10.1016\/S0140-6736(16)31408-8","volume":"388","author":"RS Finkel","year":"2016","unstructured":"Finkel RS, Chiriboga CA, Vajsar J, Day JW, Montes J, De Vivo DC, Yamashita M, Rigo F, Hung G, Schneider E, Norris DA, Xia S, Bennett CF, Bishop KM (2016) Treatment of infantile-onset spinal muscular atrophy with nusinersen: a phase 2, open-label, dose-escalation study. Lancet 388:3017\u20133026","journal-title":"Lancet"},{"key":"10_CR71","doi-asserted-by":"publisher","first-page":"1723","DOI":"10.1056\/NEJMoa1702752","volume":"377","author":"RS Finkel","year":"2017","unstructured":"Finkel RS et al (2017) Nusinersen versus sham control in infantile-onset spinal muscular atrophy. N Engl J Med 377:1723\u20131732","journal-title":"N Engl J Med"},{"key":"10_CR72","doi-asserted-by":"publisher","first-page":"139","DOI":"10.1016\/j.neulet.2011.04.047","volume":"497","author":"R Flomen","year":"2011","unstructured":"Flomen R, Makoff A (2011) Increased RNA editing in EAAT2 pre-mRNA from amyotrophic lateral sclerosis patients: involvement of a cryptic polyadenylation site. Neurosci Lett 497:139\u2013143","journal-title":"Neurosci Lett"},{"key":"10_CR73","doi-asserted-by":"publisher","first-page":"231","DOI":"10.1111\/j.1365-2990.2012.01282.x","volume":"39","author":"MC Focant","year":"2013","unstructured":"Focant MC, Goursaud S, Boucherie C, Dumont AO, Hermans E (2013) PICK1 expression in reactive astrocytes within the spinal cord of amyotrophic lateral sclerosis (ALS) rats. Neuropathol Appl Neurobiol 39:231\u2013242","journal-title":"Neuropathol Appl Neurobiol"},{"key":"10_CR74","doi-asserted-by":"publisher","first-page":"1241","DOI":"10.1002\/glia.22677","volume":"62","author":"E Foran","year":"2014","unstructured":"Foran E, Rosenblum L, Bogush A, Pasinelli P, Trotti D (2014) Sumoylation of the astroglial glutamate transporter EAAT2 governs its intracellular compartmentalization. Glia 62:1241\u20131253","journal-title":"Glia"},{"key":"10_CR75","doi-asserted-by":"publisher","first-page":"1719","DOI":"10.1002\/glia.21218","volume":"59","author":"E Foran","year":"2011","unstructured":"Foran E, Bogush A, Goffredo M, Roncaglia P, Gustincich S, Pasinelli P, Trotti D (2011) Motor neuron impairment mediated by a sumoylated fragment of the glial glutamate transporter EAAT2. Glia 59:1719\u20131731","journal-title":"Glia"},{"key":"10_CR76","doi-asserted-by":"publisher","first-page":"623","DOI":"10.1007\/s00401-011-0805-3","volume":"121","author":"K Forsberg","year":"2011","unstructured":"Forsberg K, Andersen PM, Marklund SL, Brannstrom T (2011) Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis. Acta Neuropathol 121:623\u2013634","journal-title":"Acta Neuropathol"},{"key":"10_CR77","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1038\/nbt.1515","volume":"27","author":"KD Foust","year":"2009","unstructured":"Foust KD, Nurre E, Montgomery CL, Hernandez A, Chan CM, Kaspar BK (2009) Intravascular AAV9 preferentially targets neonatal neurons and adult astrocytes. Nat Biotechnol 27:59\u201365","journal-title":"Nat Biotechnol"},{"key":"10_CR78","doi-asserted-by":"publisher","first-page":"271","DOI":"10.1038\/nbt.1610","volume":"28","author":"KD Foust","year":"2010","unstructured":"Foust KD, Wang X, McGovern VL, Braun L, Bevan AK, Haidet AM, Le TT, Morales PR, Rich MM, Burghes AH, Kaspar BK (2010) Rescue of the spinal muscular atrophy phenotype in a mouse model by early postnatal delivery of SMN. Nat Biotechnol 28:271\u2013274","journal-title":"Nat Biotechnol"},{"key":"10_CR79","doi-asserted-by":"publisher","first-page":"1009","DOI":"10.1016\/j.neuron.2014.01.013","volume":"81","author":"AE Frakes","year":"2014","unstructured":"Frakes AE, Ferraiuolo L, Haidet-Phillips AM, Schmelzer L, Braun L, Miranda CJ, Ladner KJ, Bevan AK, Foust KD, Godbout JP, Popovich PG, Guttridge DC, Kaspar BK (2014) Microglia induce motor neuron death via the classical NF-kappaB pathway in amyotrophic lateral sclerosis. Neuron 81:1009\u20131023","journal-title":"Neuron"},{"key":"10_CR80","doi-asserted-by":"publisher","first-page":"631","DOI":"10.1038\/nn.4000","volume":"18","author":"A Freischmidt","year":"2015","unstructured":"Freischmidt A et al (2015) Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia. Nat Neurosci 18:631\u2013636","journal-title":"Nat Neurosci"},{"key":"10_CR81","doi-asserted-by":"crossref","unstructured":"Fritz E, Izaurieta P, Weiss A, Mir FR, Rojas P, Gonzalez D, Rojas F, Brown RH, Madrid R, van Zundert B (2013) Mutant SOD1-expressing astrocytes release toxic factors that trigger motor neuron death by inducing hyper-excitability. J Neurophysiol","DOI":"10.1152\/jn.00500.2012"},{"key":"10_CR82","doi-asserted-by":"publisher","first-page":"279","DOI":"10.1186\/1742-2094-9-279","volume":"9","author":"AM Fukuda","year":"2012","unstructured":"Fukuda AM, Badaut J (2012) Aquaporin 4: a player in cerebral edema and neuroinflammation. J Neuroinf 9:279","journal-title":"J Neuroinf"},{"key":"10_CR83","doi-asserted-by":"publisher","first-page":"2931","DOI":"10.15252\/embj.201797568","volume":"36","author":"FB Gao","year":"2017","unstructured":"Gao FB, Almeida S, Lopez-Gonzalez R (2017) Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder. EMBO J 36:2931\u20132950","journal-title":"EMBO J"},{"key":"10_CR84","doi-asserted-by":"publisher","first-page":"126","DOI":"10.1016\/j.brainres.2007.04.044","volume":"1157","author":"S Garbuzova-Davis","year":"2007","unstructured":"Garbuzova-Davis S, Haller E, Saporta S, Kolomey I, Nicosia SV, Sanberg PR (2007) Ultrastructure of blood-brain barrier and blood-spinal cord barrier in SOD1 mice modeling ALS. Brain Res 1157:126\u2013137","journal-title":"Brain Res"},{"key":"10_CR85","doi-asserted-by":"publisher","first-page":"e1205","DOI":"10.1371\/journal.pone.0001205","volume":"2","author":"S Garbuzova-Davis","year":"2007","unstructured":"Garbuzova-Davis S, Saporta S, Haller E, Kolomey I, Bennett SP, Potter H, Sanberg PR (2007) Evidence of compromised blood-spinal cord barrier in early and late symptomatic SOD1 mice modeling ALS. PLoS ONE 2:e1205","journal-title":"PLoS ONE"},{"key":"10_CR86","doi-asserted-by":"publisher","first-page":"114","DOI":"10.1016\/j.brainres.2012.05.056","volume":"1469","author":"S Garbuzova-Davis","year":"2012","unstructured":"Garbuzova-Davis S, Hernandez-Ontiveros DG, Rodrigues MC, Haller E, Frisina-Deyo A, Mirtyl S, Sallot S, Saporta S, Borlongan CV, Sanberg PR (2012) Impaired blood-brain\/spinal cord barrier in ALS patients. Brain Res 1469:114\u2013128","journal-title":"Brain Res"},{"key":"10_CR87","doi-asserted-by":"publisher","first-page":"475","DOI":"10.1007\/s00401-004-0825-3","volume":"107","author":"MA Garcia-Cabezas","year":"2004","unstructured":"Garcia-Cabezas MA, Garcia-Alix A, Martin Y, Gutierrez M, Hernandez C, Rodriguez JI, Morales C (2004) Neonatal spinal muscular atrophy with multiple contractures, bone fractures, respiratory insufficiency and 5q13 deletion. Acta Neuropathol 107:475\u2013478","journal-title":"Acta Neuropathol"},{"key":"10_CR88","doi-asserted-by":"publisher","first-page":"134","DOI":"10.1016\/j.neubiorev.2016.02.013","volume":"64","author":"B Garcia Bueno","year":"2016","unstructured":"Garcia Bueno B, Caso JR, Madrigal JL, Leza JC (2016) Innate immune receptor Toll-like receptor 4 signalling in neuropsychiatric diseases. Neurosci Biobehav Rev 64:134\u2013147","journal-title":"Neurosci Biobehav Rev"},{"key":"10_CR89","doi-asserted-by":"publisher","first-page":"32480","DOI":"10.1074\/jbc.M704314200","volume":"282","author":"SL Gibb","year":"2007","unstructured":"Gibb SL, Boston-Howes W, Lavina ZS, Gustincich S, Brown RH Jr, Pasinelli P, Trotti D (2007) A caspase-3-cleaved fragment of the glial glutamate transporter EAAT2 is sumoylated and targeted to promyelocytic leukemia nuclear bodies in mutant SOD1-linked amyotrophic lateral sclerosis. J Biol Chem 282:32480\u201332490","journal-title":"J Biol Chem"},{"key":"10_CR90","doi-asserted-by":"publisher","first-page":"376","DOI":"10.1016\/j.bbrc.2011.11.121","volume":"417","author":"JJ Glascock","year":"2012","unstructured":"Glascock JJ, Shababi M, Wetz MJ, Krogman MM, Lorson CL (2012) Direct central nervous system delivery provides enhanced protection following vector mediated gene replacement in a severe model of spinal muscular atrophy. Biochem Biophys Res Commun 417:376\u2013381","journal-title":"Biochem Biophys Res Commun"},{"key":"10_CR91","doi-asserted-by":"publisher","first-page":"330","DOI":"10.1089\/hum.2011.166","volume":"23","author":"JJ Glascock","year":"2012","unstructured":"Glascock JJ, Osman EY, Wetz MJ, Krogman MM, Shababi M, Lorson CL (2012) Decreasing disease severity in symptomatic, Smn(-\/-);SMN2(+\/+), spinal muscular atrophy mice following scAAV9-SMN delivery. Hum Gene Ther 23:330\u2013335","journal-title":"Hum Gene Ther"},{"key":"10_CR92","doi-asserted-by":"publisher","first-page":"3818","DOI":"10.1523\/JNEUROSCI.5775-11.2012","volume":"32","author":"RG Gogliotti","year":"2012","unstructured":"Gogliotti RG, Quinlan KA, Barlow CB, Heier CR, Heckman CJ, Didonato CJ (2012) Motor neuron rescue in spinal muscular atrophy mice demonstrates that sensory-motor defects are a consequence, not a cause, of motor neuron dysfunction. J Neurosci: Off J Soc Neurosci 32:3818\u20133829","journal-title":"J Neurosci: Off J Soc Neurosci"},{"key":"10_CR93","doi-asserted-by":"publisher","first-page":"660","DOI":"10.1523\/JNEUROSCI.20-02-00660.2000","volume":"20","author":"YH Gong","year":"2000","unstructured":"Gong YH, Parsadanian AS, Andreeva A, Snider WD, Elliott JL (2000) Restricted expression of G86R Cu\/Zn superoxide dismutase in astrocytes results in astrocytosis but does not cause motoneuron degeneration. J Neurosci: Off J Soc Neurosci 20:660\u2013665","journal-title":"J Neurosci: Off J Soc Neurosci"},{"key":"10_CR94","doi-asserted-by":"publisher","first-page":"52","DOI":"10.1016\/j.mcn.2016.08.004","volume":"76","author":"A Goode","year":"2016","unstructured":"Goode A, Rea S, Sultana M, Shaw B, Searle MS, Layfield R (2016) ALS-FTLD associated mutations of SQSTM1 impact on Keap1-Nrf2 signalling. Mol Cell Neurosci 76:52\u201358","journal-title":"Mol Cell Neurosci"},{"key":"10_CR95","doi-asserted-by":"publisher","first-page":"411","DOI":"10.1038\/ng1742","volume":"38","author":"MJ Greenway","year":"2006","unstructured":"Greenway MJ, Andersen PM, Russ C, Ennis S, Cashman S, Donaghy C, Patterson V, Swingler R, Kieran D, Prehn J, Morrison KE, Green A, Acharya KR, Brown RH Jr, Hardiman O (2006) ANG mutations segregate with familial and `sporadic\u2019 amyotrophic lateral sclerosis. Nat Genet 38:411\u2013413","journal-title":"Nat Genet"},{"key":"10_CR96","doi-asserted-by":"publisher","first-page":"214","DOI":"10.1038\/nrneurol.2018.4","volume":"14","author":"EJN Groen","year":"2018","unstructured":"Groen EJN, Talbot K, Gillingwater TH (2018) Advances in therapy for spinal muscular atrophy: promises and challenges. Nat Rev Neurol 14:214\u2013224","journal-title":"Nat Rev Neurol"},{"key":"10_CR97","doi-asserted-by":"publisher","first-page":"505","DOI":"10.1016\/S1474-4422(17)30115-1","volume":"16","author":"Group W, Group EM-AS","year":"2017","unstructured":"Group W, Group EM-AS (2017) Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial. Lancet Neurol 16:505\u2013512","journal-title":"Lancet Neurol"},{"key":"10_CR98","doi-asserted-by":"publisher","first-page":"427","DOI":"10.3389\/fncel.2017.00427","volume":"11","author":"S Guerra-Gomes","year":"2017","unstructured":"Guerra-Gomes S, Sousa N, Pinto L, Oliveira JF (2017) Functional roles of astrocyte calcium elevations: from synapses to behavior. Front Cell Neurosci 11:427","journal-title":"Front Cell Neurosci"},{"key":"10_CR99","doi-asserted-by":"publisher","first-page":"546","DOI":"10.1006\/mcne.2002.1198","volume":"21","author":"H Guo","year":"2002","unstructured":"Guo H, Lai L, Butchbach ME, Lin CL (2002) Human glioma cells and undifferentiated primary astrocytes that express aberrant EAAT2 mRNA inhibit normal EAAT2 protein expression and prevent cell death. Mol Cell Neurosci 21:546\u2013560","journal-title":"Mol Cell Neurosci"},{"key":"10_CR100","doi-asserted-by":"publisher","first-page":"2519","DOI":"10.1093\/hmg\/ddg267","volume":"12","author":"H Guo","year":"2003","unstructured":"Guo H, Lai L, Butchbach ME, Stockinger MP, Shan X, Bishop GA, Lin CL (2003) Increased expression of the glial glutamate transporter EAAT2 modulates excitotoxicity and delays the onset but not the outcome of ALS in mice. Hum Mol Genet 12:2519\u20132532","journal-title":"Hum Mol Genet"},{"key":"10_CR101","doi-asserted-by":"crossref","unstructured":"Guo Y, Zhang Y, Wen D, Duan W, An T, Shi P, Wang J, Li Z, Chen X, Li C (2013) The modest impact of transcription factor Nrf2 on the course of disease in an ALS animal model. Lab Investig; J Tech Methods Pathol 93:825\u2013833","DOI":"10.1038\/labinvest.2013.73"},{"key":"10_CR102","doi-asserted-by":"publisher","first-page":"1772","DOI":"10.1126\/science.8209258","volume":"264","author":"ME Gurney","year":"1994","unstructured":"Gurney ME, Pu H, Chiu AY, Dal Canto MC, Polchow CY, Alexander DD, Caliendo J, Hentati A, Kwon YW, Deng HX et al (1994) Motor neuron degeneration in mice that express a human Cu, Zn superoxide dismutase mutation. Science 264:1772\u20131775","journal-title":"Science"},{"key":"10_CR103","doi-asserted-by":"publisher","first-page":"824","DOI":"10.1038\/nbt.1957","volume":"29","author":"AM Haidet-Phillips","year":"2011","unstructured":"Haidet-Phillips AM, Hester ME, Miranda CJ, Meyer K, Braun L, Frakes A, Song S, Likhite S, Murtha MJ, Foust KD, Rao M, Eagle A, Kammesheidt A, Christensen A, Mendell JR, Burghes AH, Kaspar BK (2011) Astrocytes from familial and sporadic ALS patients are toxic to motor neurons. Nat Biotechnol 29:824\u2013828","journal-title":"Nat Biotechnol"},{"key":"10_CR104","doi-asserted-by":"publisher","first-page":"1739","DOI":"10.1016\/j.celrep.2017.05.024","volume":"19","author":"CE Hall","year":"2017","unstructured":"Hall CE et al (2017) Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS. Cell reports 19:1739\u20131749","journal-title":"Cell reports"},{"key":"10_CR105","doi-asserted-by":"publisher","first-page":"249","DOI":"10.1002\/(SICI)1098-1136(199807)23:3<249::AID-GLIA7>3.0.CO;2-#","volume":"23","author":"ED Hall","year":"1998","unstructured":"Hall ED, Oostveen JA, Gurney ME (1998) Relationship of microglial and astrocytic activation to disease onset and progression in a transgenic model of familial ALS. Glia 23:249\u2013256","journal-title":"Glia"},{"key":"10_CR106","doi-asserted-by":"publisher","first-page":"17085","DOI":"10.1038\/nrdp.2017.85","volume":"3","author":"O Hardiman","year":"2017","unstructured":"Hardiman O, Al-Chalabi A, Chio A, Corr EM, Logroscino G, Robberecht W, Shaw PJ, Simmons Z, van den Berg LH (2017) Amyotrophic lateral sclerosis. Nat Rev Dis Prim 3:17085","journal-title":"Nat Rev Dis Prim"},{"key":"10_CR107","doi-asserted-by":"publisher","first-page":"3482","DOI":"10.1128\/MCB.21.10.3482-3490.2001","volume":"21","author":"DC Hay","year":"2001","unstructured":"Hay DC, Kemp GD, Dargemont C, Hay RT (2001) Interaction between hnRNPA1 and IkappaBalpha is required for maximal activation of NF-kappaB-dependent transcription. Mol Cell Biol 21:3482\u20133490","journal-title":"Mol Cell Biol"},{"key":"10_CR108","doi-asserted-by":"publisher","first-page":"67","DOI":"10.1002\/ajmg.1320290109","volume":"29","author":"R Herva","year":"1988","unstructured":"Herva R, Conradi NG, Kalimo H, Leisti J, Sourander P (1988) A syndrome of multiple congenital contractures: neuropathological analysis on five fetal cases. Am J Med Genet 29:67\u201376","journal-title":"Am J Med Genet"},{"key":"10_CR109","doi-asserted-by":"publisher","first-page":"610","DOI":"10.1111\/jnc.13763","volume":"139","author":"L Heyburn","year":"2016","unstructured":"Heyburn L, Hebron ML, Smith J, Winston C, Bechara J, Li Z, Lonskaya I, Burns MP, Harris BT, Moussa CE (2016) Tyrosine kinase inhibition reverses TDP-43 effects on synaptic protein expression, astrocytic function and amino acid dis-homeostasis. J Neurochem 139:610\u2013623","journal-title":"J Neurochem"},{"key":"10_CR110","doi-asserted-by":"publisher","first-page":"510","DOI":"10.1016\/j.cell.2008.02.046","volume":"133","author":"C Hochstim","year":"2008","unstructured":"Hochstim C, Deneen B, Lukaszewicz A, Zhou Q, Anderson DJ (2008) Identification of positionally distinct astrocyte subtypes whose identities are specified by a homeodomain code. Cell 133:510\u2013522","journal-title":"Cell"},{"key":"10_CR111","doi-asserted-by":"publisher","first-page":"1082","DOI":"10.1212\/WNL.55.8.1082","volume":"55","author":"LS Honig","year":"2000","unstructured":"Honig LS, Chambliss DD, Bigio EH, Carroll SL, Elliott JL (2000) Glutamate transporter EAAT2 splice variants occur not only in ALS, but also in AD and controls. Neurology 55:1082\u20131088","journal-title":"Neurology"},{"key":"10_CR112","doi-asserted-by":"publisher","first-page":"1604","DOI":"10.1073\/pnas.032539299","volume":"99","author":"DS Howland","year":"2002","unstructured":"Howland DS, Liu J, She Y, Goad B, Maragakis NJ, Kim B, Erickson J, Kulik J, DeVito L, Psaltis G, DeGennaro LJ, Cleveland DW, Rothstein JD (2002) Focal loss of the glutamate transporter EAAT2 in a transgenic rat model of SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS). Proc Natl Acad Sci USA 99:1604\u20131609","journal-title":"Proc Natl Acad Sci USA"},{"key":"10_CR113","doi-asserted-by":"publisher","first-page":"932","DOI":"10.1111\/jnc.12660","volume":"129","author":"C Huang","year":"2014","unstructured":"Huang C, Huang B, Bi F, Yan LH, Tong J, Huang J, Xia XG, Zhou H (2014) Profiling the genes affected by pathogenic TDP-43 in astrocytes. J Neurochem 129:932\u2013939","journal-title":"J Neurochem"},{"key":"10_CR114","doi-asserted-by":"publisher","first-page":"80","DOI":"10.1016\/j.nbd.2016.01.021","volume":"97","author":"CF Ib\u00e1\u00f1ez","year":"2017","unstructured":"Ib\u00e1\u00f1ez CF, Andressoo J-O (2017) Biology of GDNF and its receptors \u2014 Relevance for disorders of the central nervous system. Neurobiol Dis 97:80\u201389","journal-title":"Neurobiol Dis"},{"key":"10_CR115","doi-asserted-by":"publisher","first-page":"59","DOI":"10.3233\/JND-170209","volume":"4","author":"CC Iyer","year":"2017","unstructured":"Iyer CC, Wang X, Renusch SR, Duque SI, Wehr AM, Mo XM, McGovern VL, Arnold WD, Burghes AH, Kolb SJ (2017) SMN blood levels in a porcine model of spinal muscular atrophy. J Neuromuscul Dis 4:59\u201366","journal-title":"J Neuromuscul Dis"},{"key":"10_CR116","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1186\/s13287-018-0890-5","volume":"9","author":"M Izrael","year":"2018","unstructured":"Izrael M, Slutsky SG, Admoni T, Cohen L, Granit A, Hasson A, Itskovitz-Eldor J, Krush Paker L, Kuperstein G, Lavon N, Yehezkel Ionescu S, Solmesky LJ, Zaguri R, Zhuravlev A, Volman E, Chebath J, Revel M (2018) Safety and efficacy of human embryonic stem cell-derived astrocytes following intrathecal transplantation in SOD1(G93A) and NSG animal models. Stem Cell Res Ther 9:152","journal-title":"Stem Cell Res Ther"},{"key":"10_CR117","doi-asserted-by":"publisher","first-page":"1140","DOI":"10.1007\/s004150050532","volume":"246","author":"M Jackson","year":"1999","unstructured":"Jackson M, Steers G, Leigh PN, Morrison KE (1999) Polymorphisms in the glutamate transporter gene EAAT2 in European ALS patients. J Neurol 246:1140\u20131144","journal-title":"J Neurol"},{"key":"10_CR118","doi-asserted-by":"publisher","first-page":"135","DOI":"10.1016\/j.neubiorev.2014.12.006","volume":"49","author":"MK Jha","year":"2015","unstructured":"Jha MK, Lee S, Park DH, Kook H, Park KG, Lee IK, Suk K (2015) Diverse functional roles of lipocalin-2 in the central nervous system. Neurosci Biobehav Rev 49:135\u2013156","journal-title":"Neurosci Biobehav Rev"},{"key":"10_CR119","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1016\/j.jns.2007.01.057","volume":"255","author":"A Johansson","year":"2007","unstructured":"Johansson A, Engler H, Blomquist G, Scott B, Wall A, Aquilonius SM, Langstrom B, Askmark H (2007) Evidence for astrocytosis in ALS demonstrated by [11C](L)-deprenyl-D2 PET. J Neurol Sci 255:17\u201322","journal-title":"J Neurol Sci"},{"key":"10_CR120","doi-asserted-by":"publisher","first-page":"857","DOI":"10.1016\/j.neuron.2010.11.036","volume":"68","author":"JO Johnson","year":"2010","unstructured":"Johnson JO et al (2010) Exome sequencing reveals VCP mutations as a cause of familial ALS. Neuron 68:857\u2013864","journal-title":"Neuron"},{"key":"10_CR121","doi-asserted-by":"publisher","first-page":"664","DOI":"10.1038\/nn.3688","volume":"17","author":"JO Johnson","year":"2014","unstructured":"Johnson JO et al (2014) Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis. Nat Neurosci 17:664\u2013666","journal-title":"Nat Neurosci"},{"key":"10_CR122","doi-asserted-by":"publisher","first-page":"e0171418","DOI":"10.1371\/journal.pone.0171418","volume":"12","author":"A Jovicic","year":"2017","unstructured":"Jovicic A, Gitler AD (2017) Distinct repertoires of microRNAs present in mouse astrocytes compared to astrocyte-secreted exosomes. PLoS ONE 12:e0171418","journal-title":"PLoS ONE"},{"key":"10_CR123","doi-asserted-by":"publisher","first-page":"572","DOI":"10.1038\/ng.132","volume":"40","author":"E Kabashi","year":"2008","unstructured":"Kabashi E, Valdmanis PN, Dion P, Spiegelman D, McConkey BJ, Vande Velde C, Bouchard JP, Lacomblez L, Pochigaeva K, Salachas F, Pradat PF, Camu W, Meininger V, Dupre N, Rouleau GA (2008) TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis. Nat Genet 40:572\u2013574","journal-title":"Nat Genet"},{"key":"10_CR124","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1038\/nrrheum.2015.169","volume":"12","author":"GD Kalliolias","year":"2016","unstructured":"Kalliolias GD, Ivashkiv LB (2016) TNF biology, pathogenic mechanisms and emerging therapeutic strategies. Nat Rev Rheumatol 12:49\u201362","journal-title":"Nat Rev Rheumatol"},{"key":"10_CR125","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1007\/BF00688335","volume":"74","author":"H Kamo","year":"1987","unstructured":"Kamo H, Haebara H, Akiguchi I, Kameyama M, Kimura H, McGeer PL (1987) A distinctive distribution of reactive astroglia in the precentral cortex in amyotrophic lateral sclerosis. Acta Neuropathol 74:33\u201338","journal-title":"Acta Neuropathol"},{"key":"10_CR126","doi-asserted-by":"publisher","first-page":"1280","DOI":"10.1128\/MCB.01176-13","volume":"34","author":"P Karki","year":"2014","unstructured":"Karki P, Webb A, Smith K, Johnson J Jr, Lee K, Son DS, Aschner M, Lee E (2014) Yin Yang 1 is a repressor of glutamate transporter EAAT2, and it mediates manganese-induced decrease of EAAT2 expression in astrocytes. Mol Cell Biol 34:1280\u20131289","journal-title":"Mol Cell Biol"},{"key":"10_CR127","first-page":"973","volume":"14","author":"S Kato","year":"1999","unstructured":"Kato S, Saito M, Hirano A, Ohama E (1999) Recent advances in research on neuropathological aspects of familial amyotrophic lateral sclerosis with superoxide dismutase 1 gene mutations: neuronal Lewy body-like hyaline inclusions and astrocytic hyaline inclusions. Histol Histopathol 14:973\u2013989","journal-title":"Histol Histopathol"},{"key":"10_CR128","doi-asserted-by":"publisher","first-page":"2331","DOI":"10.1523\/JNEUROSCI.2689-13.2014","volume":"34","author":"H Kawamata","year":"2014","unstructured":"Kawamata H, Ng SK, Diaz N, Burstein S, Morel L, Osgood A, Sider B, Higashimori H, Haydon PG, Manfredi G, Yang Y (2014) Abnormal intracellular calcium signaling and SNARE-dependent exocytosis contributes to SOD1G93A astrocyte-mediated toxicity in amyotrophic lateral sclerosis. J Neurosci: Off J Soc Neurosci 34:2331\u20132348","journal-title":"J Neurosci: Off J Soc Neurosci"},{"key":"10_CR129","doi-asserted-by":"publisher","first-page":"516","DOI":"10.1002\/glia.20632","volume":"56","author":"S Keiner","year":"2008","unstructured":"Keiner S, Wurm F, Kunze A, Witte OW, Redecker C (2008) Rehabilitative therapies differentially alter proliferation and survival of glial cell populations in the perilesional zone of cortical infarcts. Glia 56:516\u2013527","journal-title":"Glia"},{"key":"10_CR130","unstructured":"Kelley KW, Ben Haim L, Schirmer L, Tyzack GE, Tolman M, Miller JG, Tsai HH, Chang SM, Molofsky AV, Yang Y, Patani R, Lakatos A, Ullian EM, Rowitch DH (2018) Kir4.1-dependent astrocyte-fast motor neuron interactions are required for peak strength. Neuron 98:306\u2013319 e307"},{"key":"10_CR131","doi-asserted-by":"publisher","first-page":"7551","DOI":"10.1038\/s41598-018-25767-0","volume":"8","author":"Y Khalfallah","year":"2018","unstructured":"Khalfallah Y, Kuta R, Grasmuck C, Prat A, Durham HD, Vande Velde C (2018) TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types. Sci Rep 8:7551","journal-title":"Sci Rep"},{"key":"10_CR132","doi-asserted-by":"publisher","first-page":"1016","DOI":"10.1002\/glia.23298","volume":"66","author":"A Kia","year":"2018","unstructured":"Kia A, McAvoy K, Krishnamurthy K, Trotti D, Pasinelli P (2018) Astrocytes expressing ALS-linked mutant FUS induce motor neuron death through release of tumor necrosis factor-alpha. Glia 66:1016\u20131033","journal-title":"Glia"},{"key":"10_CR133","doi-asserted-by":"publisher","first-page":"467","DOI":"10.1038\/nature11922","volume":"495","author":"HJ Kim","year":"2013","unstructured":"Kim HJ et al (2013) Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS. Nature 495:467\u2013473","journal-title":"Nature"},{"key":"10_CR134","doi-asserted-by":"publisher","first-page":"1587","DOI":"10.1089\/ars.2016.6966","volume":"28","author":"MJ Kim","year":"2018","unstructured":"Kim MJ, Vargas MR, Harlan BA, Killoy KM, Ball LE, Comte-Walters S, Gooz M, Yamamoto Y, Beckman JS, Barbeito L, Pehar M (2018) Nitration and glycation turn mature NGF into a toxic factor for motor neurons: a role for p75(NTR) and RAGE signaling in ALS. Antioxid Redox Signal 28:1587\u20131602","journal-title":"Antioxid Redox Signal"},{"key":"10_CR135","doi-asserted-by":"publisher","first-page":"1686","DOI":"10.1093\/brain\/awh503","volume":"128","author":"J Kirby","year":"2005","unstructured":"Kirby J, Halligan E, Baptista MJ, Allen S, Heath PR, Holden H, Barber SC, Loynes CA, Wood-Allum CA, Lunec J, Shaw PJ (2005) Mutant SOD1 alters the motor neuronal transcriptome: implications for familial ALS. Brain: J Neurol 128:1686\u20131706","journal-title":"Brain: J Neurol"},{"key":"10_CR136","doi-asserted-by":"crossref","unstructured":"Komine O, Yamashita H, Fujimori-Tonou N, Koike M, Jin S, Moriwaki Y, Endo F, Watanabe S, Uematsu S, Akira S, Uchiyama Y, Takahashi R, Misawa H, Yamanaka K (2018) Innate immune adaptor TRIF deficiency accelerates disease progression of ALS mice with accumulation of aberrantly activated astrocytes. Cell Death Differ","DOI":"10.1038\/s41418-018-0098-3"},{"key":"10_CR137","doi-asserted-by":"publisher","first-page":"242","DOI":"10.1016\/j.stemcr.2014.05.017","volume":"3","author":"T Kondo","year":"2014","unstructured":"Kondo T, Funayama M, Tsukita K, Hotta A, Yasuda A, Nori S, Kaneko S, Nakamura M, Takahashi R, Okano H, Yamanaka S, Inoue H (2014) Focal transplantation of human iPSC-derived glial-rich neural progenitors improves lifespan of ALS mice. Stem Cell Rep 3:242\u2013249","journal-title":"Stem Cell Rep"},{"key":"10_CR138","doi-asserted-by":"publisher","first-page":"e0188912","DOI":"10.1371\/journal.pone.0188912","volume":"12","author":"NR Kondori","year":"2017","unstructured":"Kondori NR, Paul P, Robbins JP, Liu K, Hildyard JCW, Wells DJ, de Belleroche JS (2017) Characterisation of the pathogenic effects of the in vivo expression of an ALS-linked mutation in D-amino acid oxidase: Phenotype and loss of spinal cord motor neurons. PLoS ONE 12:e0188912","journal-title":"PLoS ONE"},{"key":"10_CR139","doi-asserted-by":"publisher","first-page":"1255","DOI":"10.1172\/JCI66163","volume":"124","author":"Q Kong","year":"2014","unstructured":"Kong Q, Chang LC, Takahashi K, Liu Q, Schulte DA, Lai L, Ibabao B, Lin Y, Stouffer N, Das Mukhopadhyay C, Xing X, Seyb KI, Cuny GD, Glicksman MA, Lin CL (2014) Small-molecule activator of glutamate transporter EAAT2 translation provides neuroprotection. J Clin Investig 124:1255\u20131267","journal-title":"J Clin Investig"},{"key":"10_CR140","doi-asserted-by":"publisher","first-page":"99","DOI":"10.1016\/j.nbd.2014.06.008","volume":"70","author":"HG Konig","year":"2014","unstructured":"Konig HG, Coughlan KS, Kinsella S, Breen BA, Prehn JH (2014) The BCL-2 family protein Bid is critical for pro-inflammatory signaling in astrocytes. Neurobiol Dis 70:99\u2013107","journal-title":"Neurobiol Dis"},{"key":"10_CR141","doi-asserted-by":"crossref","unstructured":"Kosuge Y, Miyagishi H, Yoneoka Y, Yoneda K, Nango H, Ishige K, Ito Y (2017) Pathophysiological role of prostaglandin E2-induced up-regulation of the EP2 receptor in motor neuron-like NSC-34 cells and lumbar motor neurons in ALS model mice. Neurochem Int","DOI":"10.1016\/j.neuint.2017.06.013"},{"key":"10_CR142","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1111\/j.1440-1789.2008.00910.x","volume":"29","author":"S Kuru","year":"2009","unstructured":"Kuru S, Sakai M, Konagaya M, Yoshida M, Hashizume Y, Saito K (2009) An autopsy case of spinal muscular atrophy type III (Kugelberg-Welander disease). Neuropathol: Off J Jpn Soc Neuropathol 29:63\u201367","journal-title":"Neuropathol: Off J Jpn Soc Neuropathol"},{"key":"10_CR143","doi-asserted-by":"publisher","first-page":"263","DOI":"10.1097\/00005072-199105000-00008","volume":"50","author":"PD Kushner","year":"1991","unstructured":"Kushner PD, Stephenson DT, Wright S (1991) Reactive astrogliosis is widespread in the subcortical white matter of amyotrophic lateral sclerosis brain. J Neuropathol Exp Neurol 50:263\u2013277","journal-title":"J Neuropathol Exp Neurol"},{"key":"10_CR144","doi-asserted-by":"publisher","first-page":"1205","DOI":"10.1126\/science.1166066","volume":"323","author":"TJ Kwiatkowski Jr","year":"2009","unstructured":"Kwiatkowski TJ Jr et al (2009) Mutations in the FUS\/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Science 323:1205\u20131208","journal-title":"Science"},{"key":"10_CR145","doi-asserted-by":"publisher","first-page":"3250","DOI":"10.1172\/JCI90607","volume":"127","author":"D Lall","year":"2017","unstructured":"Lall D, Baloh RH (2017) Microglia and C9orf72 in neuroinflammation and ALS and frontotemporal dementia. J Clin Investig 127:3250\u20133258","journal-title":"J Clin Investig"},{"key":"10_CR146","doi-asserted-by":"publisher","first-page":"120","DOI":"10.1016\/j.neulet.2017.07.033","volume":"656","author":"A Lapucci","year":"2017","unstructured":"Lapucci A, Cavone L, Buonvicino D, Felici R, Gerace E, Zwergel C, Valente S, Mai A, Chiarugi A (2017) Effect of Class II HDAC inhibition on glutamate transporter expression and survival in SOD1-ALS mice. Neurosci Lett 656:120\u2013125","journal-title":"Neurosci Lett"},{"key":"10_CR147","doi-asserted-by":"publisher","first-page":"e26812","DOI":"10.1371\/journal.pone.0026812","volume":"6","author":"CS Latimer","year":"2011","unstructured":"Latimer CS, Searcy JL, Bridges MT, Brewer LD, Popovic J, Blalock EM, Landfield PW, Thibault O, Porter NM (2011) Reversal of glial and neurovascular markers of unhealthy brain aging by exercise in middle-aged female mice. PLoS ONE 6:e26812","journal-title":"PLoS ONE"},{"key":"10_CR148","doi-asserted-by":"publisher","first-page":"e46353","DOI":"10.1371\/journal.pone.0046353","volume":"7","author":"AJ Lee","year":"2012","unstructured":"Lee AJ, Awano T, Park GH, Monani UR (2012) Limited phenotypic effects of selectively augmenting the SMN protein in the neurons of a mouse model of severe spinal muscular atrophy. PLoS ONE 7:e46353","journal-title":"PLoS ONE"},{"key":"10_CR149","doi-asserted-by":"publisher","first-page":"90","DOI":"10.1186\/s12974-015-0310-z","volume":"12","author":"JY Lee","year":"2015","unstructured":"Lee JY, Lee JD, Phipps S, Noakes PG, Woodruff TM (2015) Absence of toll-like receptor 4 (TLR4) extends survival in the hSOD1 G93A mouse model of amyotrophic lateral sclerosis. Journal of neuroinflammation 12:90","journal-title":"Journal of neuroinflammation"},{"key":"10_CR150","doi-asserted-by":"publisher","first-page":"359","DOI":"10.1016\/j.bbrc.2011.02.046","volume":"406","author":"YH Leem","year":"2011","unstructured":"Leem YH, Lee YI, Son HJ, Lee SH (2011) Chronic exercise ameliorates the neuroinflammation in mice carrying NSE\/htau23. Biochem Biophys Res Commun 406:359\u2013365","journal-title":"Biochem Biophys Res Commun"},{"key":"10_CR151","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1016\/0092-8674(95)90460-3","volume":"80","author":"S Lefebvre","year":"1995","unstructured":"Lefebvre S, Burglen L, Reboullet S, Clermont O, Burlet P, Viollet L, Benichou B, Cruaud C, Millasseau P, Zeviani M et al (1995) Identification and characterization of a spinal muscular atrophy-determining gene. Cell 80:155\u2013165","journal-title":"Cell"},{"key":"10_CR152","doi-asserted-by":"publisher","first-page":"423","DOI":"10.1016\/j.expneurol.2008.02.020","volume":"211","author":"AC Lepore","year":"2008","unstructured":"Lepore AC, Dejea C, Carmen J, Rauck B, Kerr DA, Sofroniew MV, Maragakis NJ (2008) Selective ablation of proliferating astrocytes does not affect disease outcome in either acute or chronic models of motor neuron degeneration. Exp Neurol 211:423\u2013432","journal-title":"Exp Neurol"},{"key":"10_CR153","doi-asserted-by":"publisher","first-page":"1294","DOI":"10.1038\/nn.2210","volume":"11","author":"AC Lepore","year":"2008","unstructured":"Lepore AC, Rauck B, Dejea C, Pardo AC, Rao MS, Rothstein JD, Maragakis NJ (2008) Focal transplantation-based astrocyte replacement is neuroprotective in a model of motor neuron disease. Nat Neurosci 11:1294\u20131301","journal-title":"Nat Neurosci"},{"key":"10_CR154","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1016\/j.nbd.2015.03.010","volume":"78","author":"K Li","year":"2015","unstructured":"Li K, Hala TJ, Seetharam S, Poulsen DJ, Wright MC, Lepore AC (2015) GLT1 overexpression in SOD1(G93A) mouse cervical spinal cord does not preserve diaphragm function or extend disease. Neurobiol Dis 78:12\u201323","journal-title":"Neurobiol Dis"},{"key":"10_CR155","doi-asserted-by":"publisher","first-page":"1168","DOI":"10.1016\/j.neuropharm.2010.10.016","volume":"60","author":"Y Li","year":"2011","unstructured":"Li Y, Sattler R, Yang EJ, Nunes A, Ayukawa Y, Akhtar S, Ji G, Zhang PW, Rothstein JD (2011) Harmine, a natural beta-carboline alkaloid, upregulates astroglial glutamate transporter expression. Neuropharmacology 60:1168\u20131175","journal-title":"Neuropharmacology"},{"key":"10_CR156","doi-asserted-by":"publisher","first-page":"947","DOI":"10.1002\/mus.22217","volume":"44","author":"H Liang","year":"2011","unstructured":"Liang H, Ward WF, Jang YC, Bhattacharya A, Bokov AF, Li Y, Jernigan A, Richardson A, Van Remmen H (2011) PGC-1alpha protects neurons and alters disease progression in an amyotrophic lateral sclerosis mouse model. Muscle Nerve 44:947\u2013956","journal-title":"Muscle Nerve"},{"key":"10_CR157","doi-asserted-by":"publisher","first-page":"304","DOI":"10.1002\/ana.21437","volume":"64","author":"X Liang","year":"2008","unstructured":"Liang X, Wang Q, Shi J, Lokteva L, Breyer RM, Montine TJ, Andreasson K (2008) The prostaglandin E2 EP2 receptor accelerates disease progression and inflammation in a model of amyotrophic lateral sclerosis. Ann Neurol 64:304\u2013314","journal-title":"Ann Neurol"},{"key":"10_CR158","doi-asserted-by":"crossref","unstructured":"Liddell JR (2017) Are astrocytes the predominant cell type for activation of Nrf2 in aging and neurodegeneration? Antioxidants (Basel) 6","DOI":"10.3390\/antiox6030065"},{"key":"10_CR159","doi-asserted-by":"publisher","first-page":"311","DOI":"10.1007\/s00401-018-1815-1","volume":"135","author":"S Liebner","year":"2018","unstructured":"Liebner S, Dijkhuizen RM, Reiss Y, Plate KH, Agalliu D, Constantin G (2018) Functional morphology of the blood-brain barrier in health and disease. Acta Neuropathol 135:311\u2013336","journal-title":"Acta Neuropathol"},{"key":"10_CR160","doi-asserted-by":"publisher","first-page":"589","DOI":"10.1016\/S0896-6273(00)80997-6","volume":"20","author":"CL Lin","year":"1998","unstructured":"Lin CL, Bristol LA, Jin L, Dykes-Hoberg M, Crawford T, Clawson L, Rothstein JD (1998) Aberrant RNA processing in a neurodegenerative disease: the cause for absent EAAT2, a glutamate transporter, in amyotrophic lateral sclerosis. Neuron 20:589\u2013602","journal-title":"Neuron"},{"key":"10_CR161","doi-asserted-by":"publisher","first-page":"682","DOI":"10.1007\/s12035-016-0335-z","volume":"55","author":"H Lin","year":"2018","unstructured":"Lin H, Hu H, Duan W, Liu Y, Tan G, Li Z, Deng B, Song X, Wang W, Wen D, Wang Y, Li C (2018) Intramuscular delivery of scAAV9-hIGF1 prolongs survival in the hSOD1(G93A) ALS mouse model via upregulation of D-amino acid oxidase. Mol Neurobiol 55:682\u2013695","journal-title":"Mol Neurobiol"},{"key":"10_CR162","doi-asserted-by":"publisher","first-page":"3029","DOI":"10.1172\/JCI57291","volume":"121","author":"CM Lutz","year":"2011","unstructured":"Lutz CM, Kariya S, Patruni S, Osborne MA, Liu D, Henderson CE, Li DK, Pellizzoni L, Rojas J, Valenzuela DM, Murphy AJ, Winberg ML, Monani UR (2011) Postsymptomatic restoration of SMN rescues the disease phenotype in a mouse model of severe spinal muscular atrophy. J Clin Investig 121:3029\u20133041","journal-title":"J Clin Investig"},{"issue":"808\u2013816","key":"10_CR163","first-page":"e809","volume":"95","author":"IR Mackenzie","year":"2017","unstructured":"Mackenzie IR et al (2017) TIA1 mutations in amyotrophic lateral sclerosis and frontotemporal dementia promote phase separation and alter stress granule dynamics. Neuron 95(808\u2013816):e809","journal-title":"Neuron"},{"key":"10_CR164","doi-asserted-by":"publisher","first-page":"22","DOI":"10.1186\/s13041-017-0300-4","volume":"10","author":"M Madill","year":"2017","unstructured":"Madill M, McDonagh K, Ma J, Vajda A, McLoughlin P, O\u2019Brien T, Hardiman O, Shen S (2017) Amyotrophic lateral sclerosis patient iPSC-derived astrocytes impair autophagy via non-cell autonomous mechanisms. Molecular brain 10:22","journal-title":"Molecular brain"},{"key":"10_CR165","doi-asserted-by":"publisher","first-page":"592","DOI":"10.1002\/glia.23114","volume":"65","author":"B Madji Hounoum","year":"2017","unstructured":"Madji Hounoum B, Mavel S, Coque E, Patin F, Vourc\u2019h P, Marouillat S, Nadal-Desbarats L, Emond P, Corcia P, Andres CR, Raoul C, Blasco H (2017) Wildtype motoneurons, ALS-Linked SOD1 mutation and glutamate profoundly modify astrocyte metabolism and lactate shuttling. Glia 65:592\u2013605","journal-title":"Glia"},{"key":"10_CR166","doi-asserted-by":"publisher","first-page":"1527","DOI":"10.1046\/j.1460-9568.2003.02879.x","volume":"18","author":"C Maihofner","year":"2003","unstructured":"Maihofner C, Probst-Cousin S, Bergmann M, Neuhuber W, Neundorfer B, Heuss D (2003) Expression and localization of cyclooxygenase-1 and -2 in human sporadic amyotrophic lateral sclerosis. Eur J Neurosci 18:1527\u20131534","journal-title":"Eur J Neurosci"},{"key":"10_CR167","doi-asserted-by":"publisher","first-page":"649","DOI":"10.1016\/j.stem.2008.10.001","volume":"3","author":"MC Marchetto","year":"2008","unstructured":"Marchetto MC, Muotri AR, Mu Y, Smith AM, Cezar GG, Gage FH (2008) Non-cell-autonomous effect of human SOD1 G37R astrocytes on motor neurons derived from human embryonic stem cells. Cell Stem Cell 3:649\u2013657","journal-title":"Cell Stem Cell"},{"key":"10_CR168","doi-asserted-by":"publisher","first-page":"5309","DOI":"10.1523\/JNEUROSCI.3472-16.2017","volume":"37","author":"JE Martin","year":"2017","unstructured":"Martin JE, Nguyen TT, Grunseich C, Nofziger JH, Lee PR, Fields D, Fischbeck KH, Foran E (2017) Decreased motor neuron support by SMA astrocytes due to diminished MCP1 secretion. J Neurosci: Off J Soc Neurosci 37:5309\u20135318","journal-title":"J Neurosci: Off J Soc Neurosci"},{"key":"10_CR169","doi-asserted-by":"publisher","first-page":"569","DOI":"10.1080\/21678421.2017.1349151","volume":"18","author":"S Martin","year":"2017","unstructured":"Martin S, Trevor-Jones E, Khan S, Shaw K, Marchment D, Kulka A, Ellis CE, Burman R, Turner MR, Carroll L, Mursaleen L, Leigh PN, Shaw CE, Pearce N, Stahl D, Al-Chalabi A (2017) The benefit of evolving multidisciplinary care in ALS: a diagnostic cohort survival comparison. Amyotroph Lateral Scler Front Degener 18:569\u2013575","journal-title":"Amyotroph Lateral Scler Front Degener"},{"key":"10_CR170","doi-asserted-by":"publisher","first-page":"8703","DOI":"10.1523\/JNEUROSCI.0204-12.2012","volume":"32","author":"TL Martinez","year":"2012","unstructured":"Martinez TL, Kong L, Wang X, Osborne MA, Crowder ME, Van Meerbeke JP, Xu X, Davis C, Wooley J, Goldhamer DJ, Lutz CM, Rich MM, Sumner CJ (2012) Survival motor neuron protein in motor neurons determines synaptic integrity in spinal muscular atrophy. J Neurosci: Off J Soc Neurosci 32:8703\u20138715","journal-title":"J Neurosci: Off J Soc Neurosci"},{"key":"10_CR171","doi-asserted-by":"publisher","first-page":"381964","DOI":"10.1155\/2015\/381964","volume":"2015","author":"F Martorana","year":"2015","unstructured":"Martorana F, Guidotti G, Brambilla L, Rossi D (2015) Withaferin a inhibits nuclear factor-kappaB-Dependent Pro-inflammatory and stress response pathways in the astrocytes. Neural Plast 2015:381964","journal-title":"Neural Plast"},{"key":"10_CR172","doi-asserted-by":"publisher","first-page":"826","DOI":"10.1093\/hmg\/ddr513","volume":"21","author":"F Martorana","year":"2012","unstructured":"Martorana F, Brambilla L, Valori CF, Bergamaschi C, Roncoroni C, Aronica E, Volterra A, Bezzi P, Rossi D (2012) The BH4 domain of Bcl-X(L) rescues astrocyte degeneration in amyotrophic lateral sclerosis by modulating intracellular calcium signals. Hum Mol Genet 21:826\u2013840","journal-title":"Hum Mol Genet"},{"key":"10_CR173","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1038\/nature08971","volume":"465","author":"H Maruyama","year":"2010","unstructured":"Maruyama H et al (2010) Mutations of optineurin in amyotrophic lateral sclerosis. Nature 465:223\u2013226","journal-title":"Nature"},{"key":"10_CR174","doi-asserted-by":"publisher","first-page":"1418","DOI":"10.1002\/glia.22522","volume":"61","author":"JV McGivern","year":"2013","unstructured":"McGivern JV, Patitucci TN, Nord JA, Barabas MA, Stucky CL, Ebert AD (2013) Spinal muscular atrophy astrocytes exhibit abnormal calcium regulation and reduced growth factor production. Glia 61:1418\u20131428","journal-title":"Glia"},{"key":"10_CR175","doi-asserted-by":"publisher","first-page":"438","DOI":"10.1016\/j.freeradbiomed.2013.04.018","volume":"61C","author":"RJ Mead","year":"2013","unstructured":"Mead RJ, Higginbottom A, Allen SP, Kirby J, Bennett E, Barber SC, Heath PR, Coluccia A, Patel N, Gardner I, Brancale A, Grierson AJ, Shaw PJ (2013) S[+] Apomorphine is a CNS penetrating activator of the Nrf2-ARE pathway with activity in mouse and patient fibroblast models of amyotrophic lateral sclerosis. Free Radic Biol Med 61C:438\u2013452","journal-title":"Free Radic Biol Med"},{"key":"10_CR176","doi-asserted-by":"publisher","first-page":"1713","DOI":"10.1056\/NEJMoa1706198","volume":"377","author":"JR Mendell","year":"2017","unstructured":"Mendell JR et al (2017) Single-dose gene-replacement therapy for spinal muscular atrophy. N Engl J Med 377:1713\u20131722","journal-title":"N Engl J Med"},{"key":"10_CR177","doi-asserted-by":"publisher","first-page":"315","DOI":"10.1016\/j.neulet.2006.06.009","volume":"404","author":"DM Mendonca","year":"2006","unstructured":"Mendonca DM, Chimelli L, Martinez AM (2006) Expression of ubiquitin and proteasome in motorneurons and astrocytes of spinal cords from patients with amyotrophic lateral sclerosis. Neurosci Lett 404:315\u2013319","journal-title":"Neurosci Lett"},{"key":"10_CR178","doi-asserted-by":"publisher","first-page":"625","DOI":"10.1056\/NEJMoa1710504","volume":"378","author":"E Mercuri","year":"2018","unstructured":"Mercuri E et al (2018) Nusinersen versus sham control in later-onset spinal muscular atrophy. N Engl J Med 378:625\u2013635","journal-title":"N Engl J Med"},{"key":"10_CR179","doi-asserted-by":"publisher","first-page":"477","DOI":"10.1038\/mt.2014.210","volume":"23","author":"K Meyer","year":"2015","unstructured":"Meyer K, Ferraiuolo L, Schmelzer L, Braun L, McGovern V, Likhite S, Michels O, Govoni A, Fitzgerald J, Morales P, Foust KD, Mendell JR, Burghes AH, Kaspar BK (2015) Improving single injection CSF delivery of AAV9-mediated gene therapy for SMA: a dose-response study in mice and nonhuman primates. Mol Ther: J Am Soc Gene Ther 23:477\u2013487","journal-title":"Mol Ther: J Am Soc Gene Ther"},{"key":"10_CR180","doi-asserted-by":"publisher","first-page":"829","DOI":"10.1073\/pnas.1314085111","volume":"111","author":"K Meyer","year":"2014","unstructured":"Meyer K, Ferraiuolo L, Miranda CJ, Likhite S, McElroy S, Renusch S, Ditsworth D, Lagier-Tourenne C, Smith RA, Ravits J, Burghes AH, Shaw PJ, Cleveland DW, Kolb SJ, Kaspar BK (2014) Direct conversion of patient fibroblasts demonstrates non-cell autonomous toxicity of astrocytes to motor neurons in familial and sporadic ALS. Proc Natl Acad Sci USA 111:829\u2013832","journal-title":"Proc Natl Acad Sci USA"},{"key":"10_CR181","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1016\/S0022-510X(99)00196-3","volume":"170","author":"T Meyer","year":"1999","unstructured":"Meyer T, Fromm A, Munch C, Schwalenstocker B, Fray AE, Ince PG, Stamm S, Gron G, Ludolph AC, Shaw PJ (1999) The RNA of the glutamate transporter EAAT2 is variably spliced in amyotrophic lateral sclerosis and normal individuals. J Neurol Sci 170:45\u201350","journal-title":"J Neurol Sci"},{"key":"10_CR182","doi-asserted-by":"publisher","first-page":"1314","DOI":"10.1097\/00005072-199712000-00006","volume":"56","author":"A Migheli","year":"1997","unstructured":"Migheli A, Piva R, Atzori C, Troost D, Schiffer D (1997) c-Jun, JNK\/SAPK kinases and transcription factor NF-kappa B are selectively activated in astrocytes, but not motor neurons, in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 56:1314\u20131322","journal-title":"J Neuropathol Exp Neurol"},{"key":"10_CR183","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1016\/S0304-3940(98)01001-5","volume":"261","author":"A Migheli","year":"1999","unstructured":"Migheli A, Cordera S, Bendotti C, Atzori C, Piva R, Schiffer D (1999) S-100beta protein is upregulated in astrocytes and motor neurons in the spinal cord of patients with amyotrophic lateral sclerosis. Neurosci Lett 261:25\u201328","journal-title":"Neurosci Lett"},{"key":"10_CR184","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1111\/j.1471-4159.2007.04772.x","volume":"103","author":"A Milane","year":"2007","unstructured":"Milane A, Fernandez C, Vautier S, Bensimon G, Meininger V, Farinotti R (2007) Minocycline and riluzole brain disposition: interactions with p-glycoprotein at the blood-brain barrier. J Neurochem 103:164\u2013173","journal-title":"J Neurochem"},{"key":"10_CR185","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1016\/j.neulet.2008.12.061","volume":"452","author":"A Milane","year":"2009","unstructured":"Milane A, Vautier S, Chacun H, Meininger V, Bensimon G, Farinotti R, Fernandez C (2009) Interactions between riluzole and ABCG2\/BCRP transporter. Neurosci Lett 452:12\u201316","journal-title":"Neurosci Lett"},{"key":"10_CR186","doi-asserted-by":"publisher","first-page":"166","DOI":"10.1016\/j.neulet.2010.01.078","volume":"472","author":"A Milane","year":"2010","unstructured":"Milane A, Fernandez C, Dupuis L, Buyse M, Loeffler JP, Farinotti R, Meininger V, Bensimon G (2010) P-glycoprotein expression and function are increased in an animal model of amyotrophic lateral sclerosis. Neurosci Lett 472:166\u2013170","journal-title":"Neurosci Lett"},{"key":"10_CR187","doi-asserted-by":"publisher","first-page":"443","DOI":"10.3109\/21678421.2016.1143516","volume":"17","author":"M Milosevic","year":"2016","unstructured":"Milosevic M, Bataveljic D, Nikolic L, Bijelic D, Andjus P (2016) The effect of amyotrophic lateral sclerosis-linked exogenous SOD1-G93A on electrophysiological properties and intracellular calcium in cultured rat astrocytes. Amyotroph Lateral Scler Front Degener 17:443\u2013451","journal-title":"Amyotroph Lateral Scler Front Degener"},{"key":"10_CR188","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1016\/j.brainres.2011.12.064","volume":"1446","author":"T Mimoto","year":"2012","unstructured":"Mimoto T, Miyazaki K, Morimoto N, Kurata T, Satoh K, Ikeda Y, Abe K (2012) Impaired antioxydative Keap1\/Nrf2 system and the downstream stress protein responses in the motor neuron of ALS model mice. Brain Res 1446:109\u2013118","journal-title":"Brain Res"},{"key":"10_CR189","doi-asserted-by":"publisher","first-page":"e34776","DOI":"10.1371\/journal.pone.0034776","volume":"7","author":"E Miquel","year":"2012","unstructured":"Miquel E, Cassina A, Martinez-Palma L, Bolatto C, Trias E, Gandelman M, Radi R, Barbeito L, Cassina P (2012) Modulation of astrocytic mitochondrial function by dichloroacetate improves survival and motor performance in inherited amyotrophic lateral sclerosis. PLoS ONE 7:e34776","journal-title":"PLoS ONE"},{"key":"10_CR190","doi-asserted-by":"publisher","first-page":"7556","DOI":"10.1073\/pnas.0914128107","volume":"107","author":"J Mitchell","year":"2010","unstructured":"Mitchell J, Paul P, Chen HJ, Morris A, Payling M, Falchi M, Habgood J, Panoutsou S, Winkler S, Tisato V, Hajitou A, Smith B, Vance C, Shaw C, Mazarakis ND, de Belleroche J (2010) Familial amyotrophic lateral sclerosis is associated with a mutation in D-amino acid oxidase. Proc Natl Acad Sci USA 107:7556\u20137561","journal-title":"Proc Natl Acad Sci USA"},{"key":"10_CR191","doi-asserted-by":"publisher","first-page":"445","DOI":"10.1007\/s10571-016-0377-9","volume":"37","author":"H Miyagishi","year":"2017","unstructured":"Miyagishi H, Kosuge Y, Takano A, Endo M, Nango H, Yamagata-Murayama S, Hirose D, Kano R, Tanaka Y, Ishige K, Ito Y (2017) Increased expression of 15-hydroxyprostaglandin dehydrogenase in spinal astrocytes during disease progression in a model of amyotrophic lateral sclerosis. Cell Mol Neurobiol 37:445\u2013452","journal-title":"Cell Mol Neurobiol"},{"key":"10_CR192","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1038\/nature13161","volume":"509","author":"AV Molofsky","year":"2014","unstructured":"Molofsky AV, Kelley KW, Tsai HH, Redmond SA, Chang SM, Madireddy L, Chan JR, Baranzini SE, Ullian EM, Rowitch DH (2014) Astrocyte-encoded positional cues maintain sensorimotor circuit integrity. Nature 509:189\u2013194","journal-title":"Nature"},{"key":"10_CR193","doi-asserted-by":"publisher","first-page":"7105","DOI":"10.1074\/jbc.M112.410944","volume":"288","author":"L Morel","year":"2013","unstructured":"Morel L, Regan M, Higashimori H, Ng SK, Esau C, Vidensky S, Rothstein J, Yang Y (2013) Neuronal exosomal miRNA-dependent translational regulation of astroglial glutamate transporter GLT1. J Biol Chem 288:7105\u20137116","journal-title":"J Biol Chem"},{"key":"10_CR194","doi-asserted-by":"publisher","first-page":"1732","DOI":"10.1093\/hmg\/ddx093","volume":"26","author":"D Moujalled","year":"2017","unstructured":"Moujalled D, Grubman A, Acevedo K, Yang S, Ke YD, Moujalled DM, Duncan C, Caragounis A, Perera ND, Turner BJ, Prudencio M, Petrucelli L, Blair I, Ittner LM, Crouch PJ, Liddell JR, White AR (2017) TDP-43 mutations causing amyotrophic lateral sclerosis are associated with altered expression of RNA-binding protein hnRNP K and affect the Nrf2 antioxidant pathway. Hum Mol Genet 26:1732\u20131746","journal-title":"Hum Mol Genet"},{"key":"10_CR195","doi-asserted-by":"publisher","first-page":"1655","DOI":"10.1093\/hmg\/ddu578","volume":"24","author":"D Moujalled","year":"2015","unstructured":"Moujalled D, James JL, Yang S, Zhang K, Duncan C, Moujalled DM, Parker SJ, Caragounis A, Lidgerwood G, Turner BJ, Atkin JD, Grubman A, Liddell JR, Proepper C, Boeckers TM, Kanninen KM, Blair I, Crouch PJ, White AR (2015) Phosphorylation of hnRNP K by cyclin-dependent kinase 2 controls cytosolic accumulation of TDP-43. Hum Mol Genet 24:1655\u20131669","journal-title":"Hum Mol Genet"},{"key":"10_CR196","doi-asserted-by":"publisher","first-page":"456","DOI":"10.1007\/BF00293379","volume":"82","author":"S Murayama","year":"1991","unstructured":"Murayama S, Inoue K, Kawakami H, Bouldin TW, Suzuki K (1991) A unique pattern of astrocytosis in the primary motor area in amyotrophic lateral sclerosis. Acta Neuropathol 82:456\u2013461","journal-title":"Acta Neuropathol"},{"key":"10_CR197","doi-asserted-by":"publisher","first-page":"813","DOI":"10.1212\/WNL.0000000000002305","volume":"86","author":"J Murphy","year":"2016","unstructured":"Murphy J et al (2016) Cognitive-behavioral screening reveals prevalent impairment in a large multicenter ALS cohort. Neurology 86:813\u2013820","journal-title":"Neurology"},{"key":"10_CR198","doi-asserted-by":"publisher","first-page":"615","DOI":"10.1038\/nn1876","volume":"10","author":"M Nagai","year":"2007","unstructured":"Nagai M, Re DB, Nagata T, Chalazonitis A, Jessell TM, Wichterle H, Przedborski S (2007) Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons. Nat Neurosci 10:615\u2013622","journal-title":"Nat Neurosci"},{"key":"10_CR199","doi-asserted-by":"publisher","first-page":"336","DOI":"10.1002\/jnr.490380312","volume":"38","author":"D Nagy","year":"1994","unstructured":"Nagy D, Kato T, Kushner PD (1994) Reactive astrocytes are widespread in the cortical gray matter of amyotrophic lateral sclerosis. J Neurosci Res 38:336\u2013347","journal-title":"J Neurosci Res"},{"key":"10_CR200","doi-asserted-by":"crossref","unstructured":"Nanou A, Higginbottom A, Valori CF, Wyles M, Ning K, Shaw P, Azzouz M (2013) Viral delivery of antioxidant genes as a therapeutic strategy in experimental models of amyotrophic lateral sclerosis. Mol Ther: J Am Soc Gene Ther","DOI":"10.1038\/mt.2013.115"},{"key":"10_CR201","doi-asserted-by":"publisher","first-page":"130","DOI":"10.1126\/science.1134108","volume":"314","author":"M Neumann","year":"2006","unstructured":"Neumann M, Sampathu DM, Kwong LK, Truax AC, Micsenyi MC, Chou TT, Bruce J, Schuck T, Grossman M, Clark CM, McCluskey LF, Miller BL, Masliah E, Mackenzie IR, Feldman H, Feiden W, Kretzschmar HA, Trojanowski JQ, Lee VM (2006) Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314:130\u2013133","journal-title":"Science"},{"key":"10_CR202","doi-asserted-by":"publisher","first-page":"88","DOI":"10.1016\/j.freeradbiomed.2011.03.027","volume":"51","author":"A Neymotin","year":"2011","unstructured":"Neymotin A, Calingasan NY, Wille E, Naseri N, Petri S, Damiano M, Liby KT, Risingsong R, Sporn M, Beal MF, Kiaei M (2011) Neuroprotective effect of Nrf2\/ARE activators, CDDO ethylamide and CDDO trifluoroethylamide, in a mouse model of amyotrophic lateral sclerosis. Free Radic Biol Med 51:88\u201396","journal-title":"Free Radic Biol Med"},{"key":"10_CR203","doi-asserted-by":"publisher","first-page":"207","DOI":"10.1002\/ar.20838","volume":"292","author":"C Nicaise","year":"2009","unstructured":"Nicaise C, Soyfoo MS, Authelet M, De Decker R, Bataveljic D, Delporte C, Pochet R (2009) Aquaporin-4 overexpression in rat ALS model. Anat Rec (Hoboken) 292:207\u2013213","journal-title":"Anat Rec (Hoboken)"},{"key":"10_CR204","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1186\/1742-2094-5-13","volume":"5","author":"KE Nichol","year":"2008","unstructured":"Nichol KE, Poon WW, Parachikova AI, Cribbs DH, Glabe CG, Cotman CW (2008) Exercise alters the immune profile in Tg2576 Alzheimer mice toward a response coincident with improved cognitive performance and decreased amyloid. J Neuroinflammation 5:13","journal-title":"J Neuroinflammation"},{"issue":"1268\u20131283","key":"10_CR205","first-page":"e1266","volume":"97","author":"A Nicolas","year":"2018","unstructured":"Nicolas A et al (2018) Genome-wide analyses identify KIF5A as a novel ALS gene. Neuron 97(1268\u20131283):e1266","journal-title":"Neuron"},{"key":"10_CR206","doi-asserted-by":"publisher","first-page":"223","DOI":"10.2217\/nmt.14.21","volume":"4","author":"A Nonneman","year":"2014","unstructured":"Nonneman A, Robberecht W, Van Den Bosch L (2014) The role of oligodendroglial dysfunction in amyotrophic lateral sclerosis. Neurodegener Dis Manag 4:223\u2013239","journal-title":"Neurodegener Dis Manag"},{"key":"10_CR207","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1016\/j.nbd.2018.07.012","volume":"119","author":"A Nonneman","year":"2018","unstructured":"Nonneman A, Criem N, Lewandowski SA, Nuyts R, Thal DR, Pfrieger FW, Ravits J, Van Damme P, Zwijsen A, Van Den Bosch L, Robberecht W (2018) Astrocyte-derived Jagged-1 mitigates deleterious Notch signaling in amyotrophic lateral sclerosis. Neurobiol Dis 119:26\u201340","journal-title":"Neurobiol Dis"},{"key":"10_CR208","doi-asserted-by":"publisher","first-page":"203","DOI":"10.1016\/0014-4886(95)90042-X","volume":"131","author":"SA O\u2019Reilly","year":"1995","unstructured":"O\u2019Reilly SA, Roedica J, Nagy D, Hallewell RA, Alderson K, Marklund SL, Kuby J, Kushner PD (1995) Motor neuron-astrocyte interactions and levels of Cu, Zn superoxide dismutase in sporadic amyotrophic lateral sclerosis. Exp Neurol 131:203\u2013210","journal-title":"Exp Neurol"},{"key":"10_CR209","doi-asserted-by":"crossref","unstructured":"Osipova ED, Semyachkina-Glushkovskaya OV, Morgun AV, Pisareva NV, Malinovskaya NA, Boitsova EB, Pozhilenkova EA, Belova OA, Salmin VV, Taranushenko TE, Noda M, Salmina AB (2018) Gliotransmitters and cytokines in the control of blood-brain barrier permeability. Rev Neurosci","DOI":"10.1515\/revneuro-2017-0092"},{"key":"10_CR210","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1097\/WNR.0000000000000043","volume":"25","author":"B Otsmane","year":"2014","unstructured":"Otsmane B, Aebischer J, Moumen A, Raoul C (2014) Cerebrospinal fluid-targeted delivery of neutralizing anti-IFNgamma antibody delays motor decline in an ALS mouse model. NeuroReport 25:49\u201354","journal-title":"NeuroReport"},{"key":"10_CR211","unstructured":"Ouali Alami N et al (2018) NF-kappaB activation in astrocytes drives a stage-specific beneficial neuroimmunological response in ALS. EMBO J 37"},{"key":"10_CR212","doi-asserted-by":"publisher","first-page":"e75866","DOI":"10.1371\/journal.pone.0075866","volume":"8","author":"X Paez-Colasante","year":"2013","unstructured":"Paez-Colasante X, Seaberg B, Martinez TL, Kong L, Sumner CJ, Rimer M (2013) Improvement of neuromuscular synaptic phenotypes without enhanced survival and motor function in severe spinal muscular atrophy mice selectively rescued in motor neurons. PLoS ONE 8:e75866","journal-title":"PLoS ONE"},{"key":"10_CR213","doi-asserted-by":"publisher","first-page":"17803","DOI":"10.1073\/pnas.1103141108","volume":"108","author":"ST Papadeas","year":"2011","unstructured":"Papadeas ST, Kraig SE, O\u2019Banion C, Lepore AC, Maragakis NJ (2011) Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo. Proc Natl Acad Sci USA 108:17803\u201317808","journal-title":"Proc Natl Acad Sci USA"},{"key":"10_CR214","doi-asserted-by":"publisher","first-page":"121","DOI":"10.1016\/j.nbd.2007.12.008","volume":"30","author":"A Parachikova","year":"2008","unstructured":"Parachikova A, Nichol KE, Cotman CW (2008) Short-term exercise in aged Tg2576 mice alters neuroinflammation and improves cognition. Neurobiol Dis 30:121\u2013129","journal-title":"Neurobiol Dis"},{"key":"10_CR215","doi-asserted-by":"publisher","first-page":"120","DOI":"10.1016\/j.expneurol.2006.03.028","volume":"201","author":"AC Pardo","year":"2006","unstructured":"Pardo AC, Wong V, Benson LM, Dykes M, Tanaka K, Rothstein JD, Maragakis NJ (2006) Loss of the astrocyte glutamate transporter GLT1 modifies disease in SOD1(G93A) mice. Exp Neurol 201:120\u2013130","journal-title":"Exp Neurol"},{"key":"10_CR216","doi-asserted-by":"publisher","first-page":"12005","DOI":"10.1523\/JNEUROSCI.2208-10.2010","volume":"30","author":"GH Park","year":"2010","unstructured":"Park GH, Maeno-Hikichi Y, Awano T, Landmesser LT, Monani UR (2010) Reduced survival of motor neuron (SMN) protein in motor neuronal progenitors functions cell autonomously to cause spinal muscular atrophy in model mice expressing the human centromeric (SMN2) gene. J Neurosci: Off J Soc Neurosci 30:12005\u201312019","journal-title":"J Neurosci: Off J Soc Neurosci"},{"key":"10_CR217","doi-asserted-by":"publisher","first-page":"13901","DOI":"10.1073\/pnas.240305897","volume":"97","author":"P Pasinelli","year":"2000","unstructured":"Pasinelli P, Houseweart MK, Brown RH Jr, Cleveland DW (2000) Caspase-1 and -3 are sequentially activated in motor neuron death in Cu, Zn superoxide dismutase-mediated familial amyotrophic lateral sclerosis. Proc Natl Acad Sci USA 97:13901\u201313906","journal-title":"Proc Natl Acad Sci USA"},{"key":"10_CR218","doi-asserted-by":"publisher","first-page":"217","DOI":"10.1007\/s13311-014-0311-0","volume":"12","author":"P Patel","year":"2015","unstructured":"Patel P, Julien JP, Kriz J (2015) Early-stage treatment with Withaferin A reduces levels of misfolded superoxide dismutase 1 and extends lifespan in a mouse model of amyotrophic lateral sclerosis. Neurother: J Am Soc Exp NeuroTherapeutics 12:217\u2013233","journal-title":"Neurother: J Am Soc Exp NeuroTherapeutics"},{"key":"10_CR219","doi-asserted-by":"publisher","first-page":"514","DOI":"10.1093\/hmg\/ddv489","volume":"25","author":"TN Patitucci","year":"2016","unstructured":"Patitucci TN, Ebert AD (2016) SMN deficiency does not induce oxidative stress in SMA iPSC-derived astrocytes or motor neurons. Hum Mol Genet 25:514\u2013523","journal-title":"Hum Mol Genet"},{"key":"10_CR220","doi-asserted-by":"publisher","first-page":"247","DOI":"10.1016\/j.tins.2018.03.010","volume":"41","author":"A Pattamatta","year":"2018","unstructured":"Pattamatta A, Cleary JD, Ranum LPW (2018) All in the Family: Repeats and ALS\/FTD. Trends Neurosci 41:247\u2013250","journal-title":"Trends Neurosci"},{"key":"10_CR221","doi-asserted-by":"publisher","first-page":"876","DOI":"10.1016\/j.neurobiolaging.2013.09.005","volume":"35","author":"P Paul","year":"2014","unstructured":"Paul P, Murphy T, Oseni Z, Sivalokanathan S, de Belleroche JS (2014) Pathogenic effects of amyotrophic lateral sclerosis-linked mutation in D-amino acid oxidase are mediated by D-serine. Neurobiol Aging 35:876\u2013885","journal-title":"Neurobiol Aging"},{"key":"10_CR222","doi-asserted-by":"publisher","first-page":"2879","DOI":"10.1093\/brain\/awu169","volume":"137","author":"K Peeters","year":"2014","unstructured":"Peeters K, Chamova T, Jordanova A (2014) Clinical and genetic diversity of SMN1-negative proximal spinal muscular atrophies. Brain: J Neurol 137:2879\u20132896","journal-title":"Brain: J Neurol"},{"key":"10_CR223","doi-asserted-by":"publisher","first-page":"e103438","DOI":"10.1371\/journal.pone.0103438","volume":"9","author":"M Pehar","year":"2014","unstructured":"Pehar M, Beeson G, Beeson CC, Johnson JA, Vargas MR (2014) Mitochondria-targeted catalase reverts the neurotoxicity of hSOD1G(9)(3)A astrocytes without extending the survival of ALS-linked mutant hSOD1 mice. PLoS ONE 9:e103438","journal-title":"PLoS ONE"},{"key":"10_CR224","doi-asserted-by":"publisher","first-page":"7777","DOI":"10.1523\/JNEUROSCI.0823-07.2007","volume":"27","author":"M Pehar","year":"2007","unstructured":"Pehar M, Vargas MR, Robinson KM, Cassina P, Diaz-Amarilla PJ, Hagen TM, Radi R, Barbeito L, Beckman JS (2007) Mitochondrial superoxide production and nuclear factor erythroid 2-related factor 2 activation in p75 neurotrophin receptor-induced motor neuron apoptosis. J Neurosci: Off J Soc Neurosci 27:7777\u20137785","journal-title":"J Neurosci: Off J Soc Neurosci"},{"key":"10_CR225","doi-asserted-by":"publisher","first-page":"E756","DOI":"10.1073\/pnas.1222361110","volume":"110","author":"HP Phatnani","year":"2013","unstructured":"Phatnani HP, Guarnieri P, Friedman BA, Carrasco MA, Muratet M, O\u2019Keeffe S, Nwakeze C, Pauli-Behn F, Newberry KM, Meadows SK, Tapia JC, Myers RM, Maniatis T (2013) Intricate interplay between astrocytes and motor neurons in ALS. Proc Natl Acad Sci USA 110:E756\u2013765","journal-title":"Proc Natl Acad Sci USA"},{"key":"10_CR226","doi-asserted-by":"publisher","first-page":"252","DOI":"10.1016\/j.nbd.2012.12.017","volume":"54","author":"CS Piao","year":"2013","unstructured":"Piao CS, Stoica BA, Wu J, Sabirzhanov B, Zhao Z, Cabatbat R, Loane DJ, Faden AI (2013) Late exercise reduces neuroinflammation and cognitive dysfunction after traumatic brain injury. Neurobiol Dis 54:252\u2013263","journal-title":"Neurobiol Dis"},{"key":"10_CR227","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1186\/s13041-015-0162-6","volume":"8","author":"V Picher-Martel","year":"2015","unstructured":"Picher-Martel V, Dutta K, Phaneuf D, Sobue G, Julien JP (2015) Ubiquilin-2 drives NF-kappaB activity and cytosolic TDP-43 aggregation in neuronal cells. Mol Brain 8:71","journal-title":"Mol Brain"},{"key":"10_CR228","doi-asserted-by":"publisher","first-page":"843","DOI":"10.1016\/j.stemcr.2017.03.003","volume":"8","author":"K Qian","year":"2017","unstructured":"Qian K, Huang H, Peterson A, Hu B, Maragakis NJ, Ming GL, Chen H, Zhang SC (2017) Sporadic ALS astrocytes induce neuronal degeneration in vivo. Stem Cell Rep 8:843\u2013855","journal-title":"Stem Cell Rep"},{"key":"10_CR229","doi-asserted-by":"publisher","first-page":"1298","DOI":"10.1002\/glia.23003","volume":"64","author":"H Qosa","year":"2016","unstructured":"Qosa H, Lichter J, Sarlo M, Markandaiah SS, McAvoy K, Richard JP, Jablonski MR, Maragakis NJ, Pasinelli P, Trotti D (2016) Astrocytes drive upregulation of the multidrug resistance transporter ABCB1 (P-Glycoprotein) in endothelial cells of the blood-brain barrier in mutant superoxide dismutase 1-linked amyotrophic lateral sclerosis. Glia 64:1298\u20131313","journal-title":"Glia"},{"key":"10_CR230","doi-asserted-by":"publisher","first-page":"2610","DOI":"10.1002\/jcp.25827","volume":"232","author":"UN Ramirez-Jarquin","year":"2017","unstructured":"Ramirez-Jarquin UN, Rojas F, van Zundert B, Tapia R (2017) Chronic infusion of SOD1(G93A) astrocyte-secreted factors induces spinal motoneuron degeneration and neuromuscular dysfunction in healthy rats. J Cell Physiol 232:2610\u20132615","journal-title":"J Cell Physiol"},{"key":"10_CR231","doi-asserted-by":"publisher","first-page":"1001","DOI":"10.1016\/j.neuron.2014.01.011","volume":"81","author":"DB Re","year":"2014","unstructured":"Re DB, Le Verche V, Yu C, Amoroso MW, Politi KA, Phani S, Ikiz B, Hoffmann L, Koolen M, Nagata T, Papadimitriou D, Nagy P, Mitsumoto H, Kariya S, Wichterle H, Henderson CE, Przedborski S (2014) Necroptosis drives motor neuron death in models of both sporadic and familial ALS. Neuron 81:1001\u20131008","journal-title":"Neuron"},{"key":"10_CR232","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1016\/j.neuron.2011.09.010","volume":"72","author":"AE Renton","year":"2011","unstructured":"Renton AE et al (2011) A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron 72:257\u2013268","journal-title":"Neuron"},{"key":"10_CR233","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1038\/nrneurol.2017.148","volume":"14","author":"C Rinaldi","year":"2018","unstructured":"Rinaldi C, Wood MJA (2018) Antisense oligonucleotides: the next frontier for treatment of neurological disorders. Nat Rev Neurol 14:9\u201321","journal-title":"Nat Rev Neurol"},{"key":"10_CR234","doi-asserted-by":"publisher","first-page":"4094","DOI":"10.1093\/hmg\/ddv148","volume":"24","author":"H Rindt","year":"2015","unstructured":"Rindt H, Feng Z, Mazzasette C, Glascock JJ, Valdivia D, Pyles N, Crawford TO, Swoboda KJ, Patitucci TN, Ebert AD, Sumner CJ, Ko CP, Lorson CL (2015) Astrocytes influence the severity of spinal muscular atrophy. Hum Mol Genet 24:4094\u20134102","journal-title":"Hum Mol Genet"},{"key":"10_CR235","doi-asserted-by":"publisher","first-page":"4559","DOI":"10.1093\/hmg\/ddu169","volume":"23","author":"KL Robbins","year":"2014","unstructured":"Robbins KL, Glascock JJ, Osman EY, Miller MR, Lorson CL (2014) Defining the therapeutic window in a severe animal model of spinal muscular atrophy. Hum Mol Genet 23:4559\u20134568","journal-title":"Hum Mol Genet"},{"key":"10_CR236","doi-asserted-by":"publisher","first-page":"56","DOI":"10.1016\/j.neuint.2016.03.014","volume":"98","author":"MB Robinson","year":"2016","unstructured":"Robinson MB, Jackson JG (2016) Astroglial glutamate transporters coordinate excitatory signaling and brain energetics. Neurochem Int 98:56\u201371","journal-title":"Neurochem Int"},{"key":"10_CR237","doi-asserted-by":"publisher","first-page":"e678","DOI":"10.1038\/cddis.2013.194","volume":"4","author":"JJ Rodriguez","year":"2013","unstructured":"Rodriguez JJ, Terzieva S, Olabarria M, Lanza RG, Verkhratsky A (2013) Enriched environment and physical activity reverse astrogliodegeneration in the hippocampus of AD transgenic mice. Cell Death Dis 4:e678","journal-title":"Cell Death Dis"},{"key":"10_CR238","doi-asserted-by":"publisher","first-page":"24","DOI":"10.3389\/fncel.2014.00024","volume":"8","author":"F Rojas","year":"2014","unstructured":"Rojas F, Cortes N, Abarzua S, Dyrda A, van Zundert B (2014) Astrocytes expressing mutant SOD1 and TDP43 trigger motoneuron death that is mediated via sodium channels and nitroxidative stress. Front Cell Neurosci 8:24","journal-title":"Front Cell Neurosci"},{"key":"10_CR239","doi-asserted-by":"publisher","first-page":"203","DOI":"10.3389\/fncel.2015.00203","volume":"9","author":"F Rojas","year":"2015","unstructured":"Rojas F, Gonzalez D, Cortes N, Ampuero E, Hernandez DE, Fritz E, Abarzua S, Martinez A, Elorza AA, Alvarez A, Court F, van Zundert B (2015) Reactive oxygen species trigger motoneuron death in non-cell-autonomous models of ALS through activation of c-Abl signaling. Front Cell Neurosci 9:203","journal-title":"Front Cell Neurosci"},{"key":"10_CR240","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1038\/362059a0","volume":"362","author":"DR Rosen","year":"1993","unstructured":"Rosen DR, Siddique T, Patterson D, Figlewicz DA, Sapp P, Hentati A, Donaldson D, Goto J, O\u2019Regan JP, Deng HX et al (1993) Mutations in Cu\/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 362:59\u201362","journal-title":"Nature"},{"key":"10_CR241","doi-asserted-by":"publisher","first-page":"145","DOI":"10.1016\/j.expneurol.2017.03.014","volume":"292","author":"LT Rosenblum","year":"2017","unstructured":"Rosenblum LT, Shamamandri-Markandaiah S, Ghosh B, Foran E, Lepore AC, Pasinelli P, Trotti D (2017) Mutation of the caspase-3 cleavage site in the astroglial glutamate transporter EAAT2 delays disease progression and extends lifespan in the SOD1-G93A mouse model of ALS. Exp Neurol 292:145\u2013153","journal-title":"Exp Neurol"},{"key":"10_CR242","doi-asserted-by":"publisher","first-page":"1691","DOI":"10.1038\/cdd.2008.99","volume":"15","author":"D Rossi","year":"2008","unstructured":"Rossi D, Brambilla L, Valori CF, Roncoroni C, Crugnola A, Yokota T, Bredesen DE, Volterra A (2008) Focal degeneration of astrocytes in amyotrophic lateral sclerosis. Cell Death Differ 15:1691\u20131700","journal-title":"Cell Death Differ"},{"key":"10_CR243","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1002\/ana.410380114","volume":"38","author":"JD Rothstein","year":"1995","unstructured":"Rothstein JD, Van Kammen M, Levey AI, Martin LJ, Kuncl RW (1995) Selective loss of glial glutamate transporter GLT-1 in amyotrophic lateral sclerosis. Ann Neurol 38:73\u201384","journal-title":"Ann Neurol"},{"key":"10_CR244","doi-asserted-by":"publisher","first-page":"18","DOI":"10.1002\/ana.410280106","volume":"28","author":"JD Rothstein","year":"1990","unstructured":"Rothstein JD, Tsai G, Kuncl RW, Clawson L, Cornblath DR, Drachman DB, Pestronk A, Stauch BL, Coyle JT (1990) Abnormal excitatory amino acid metabolism in amyotrophic lateral sclerosis. Ann Neurol 28:18\u201325","journal-title":"Ann Neurol"},{"key":"10_CR245","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1038\/nature03180","volume":"433","author":"JD Rothstein","year":"2005","unstructured":"Rothstein JD, Patel S, Regan MR, Haenggeli C, Huang YH, Bergles DE, Jin L, Dykes Hoberg M, Vidensky S, Chung DS, Toan SV, Bruijn LI, Su ZZ, Gupta P, Fisher PB (2005) Beta-lactam antibiotics offer neuroprotection by increasing glutamate transporter expression. Nature 433:73\u201377","journal-title":"Nature"},{"key":"10_CR246","doi-asserted-by":"publisher","first-page":"638","DOI":"10.1016\/j.tins.2012.06.001","volume":"35","author":"M Santello","year":"2012","unstructured":"Santello M, Volterra A (2012) TNFalpha in synaptic function: switching gears. Trends Neurosci 35:638\u2013647","journal-title":"Trends Neurosci"},{"key":"10_CR247","doi-asserted-by":"publisher","first-page":"e39113","DOI":"10.1371\/journal.pone.0039113","volume":"7","author":"D Sareen","year":"2012","unstructured":"Sareen D, Ebert AD, Heins BM, McGivern JV, Ornelas L, Svendsen CN (2012) Inhibition of apoptosis blocks human motor neuron cell death in a stem cell model of spinal muscular atrophy. PLoS ONE 7:e39113","journal-title":"PLoS ONE"},{"key":"10_CR248","doi-asserted-by":"publisher","first-page":"1055","DOI":"10.1097\/NEN.0b013e31818b4906","volume":"67","author":"A Sarlette","year":"2008","unstructured":"Sarlette A, Krampfl K, Grothe C, Neuhoff N, Dengler R, Petri S (2008) Nuclear erythroid 2-related factor 2-antioxidative response element signaling pathway in motor cortex and spinal cord in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 67:1055\u20131062","journal-title":"J Neuropathol Exp Neurol"},{"key":"10_CR249","doi-asserted-by":"publisher","first-page":"4149","DOI":"10.1038\/sj.emboj.7601840","volume":"26","author":"J Sasabe","year":"2007","unstructured":"Sasabe J, Chiba T, Yamada M, Okamoto K, Nishimoto I, Matsuoka M, Aiso S (2007) D-serine is a key determinant of glutamate toxicity in amyotrophic lateral sclerosis. The EMBO journal 26:4149\u20134159","journal-title":"The EMBO journal"},{"key":"10_CR250","doi-asserted-by":"publisher","first-page":"627","DOI":"10.1073\/pnas.1114639109","volume":"109","author":"J Sasabe","year":"2012","unstructured":"Sasabe J, Miyoshi Y, Suzuki M, Mita M, Konno R, Matsuoka M, Hamase K, Aiso S (2012) D-amino acid oxidase controls motoneuron degeneration through D-serine. Proc Natl Acad Sci USA 109:627\u2013632","journal-title":"Proc Natl Acad Sci USA"},{"key":"10_CR251","doi-asserted-by":"publisher","first-page":"44","DOI":"10.1016\/S0304-3940(00)01370-7","volume":"291","author":"S Sasaki","year":"2000","unstructured":"Sasaki S, Shibata N, Komori T, Iwata M (2000) iNOS and nitrotyrosine immunoreactivity in amyotrophic lateral sclerosis. Neurosci Lett 291:44\u201348","journal-title":"Neurosci Lett"},{"key":"10_CR252","doi-asserted-by":"crossref","unstructured":"Saur L, Baptista PP, de Senna PN, Paim MF, do Nascimento P, Ilha J, Bagatini PB, Achaval M, Xavier LL (2014) Physical exercise increases GFAP expression and induces morphological changes in hippocampal astrocytes. Brain structure and function 219:293\u2013302","DOI":"10.1007\/s00429-012-0500-8"},{"issue":"Suppl","key":"10_CR253","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1016\/0022-510X(96)00073-1","volume":"139","author":"D Schiffer","year":"1996","unstructured":"Schiffer D, Cordera S, Cavalla P, Migheli A (1996) Reactive astrogliosis of the spinal cord in amyotrophic lateral sclerosis. J Neurol Sci 139(Suppl):27\u201333","journal-title":"J Neurol Sci"},{"key":"10_CR254","doi-asserted-by":"publisher","first-page":"178","DOI":"10.1016\/j.neulet.2010.06.087","volume":"481","author":"TB Seo","year":"2010","unstructured":"Seo TB, Kim BK, Ko IG, Kim DH, Shin MS, Kim CJ, Yoon JH, Kim H (2010) Effect of treadmill exercise on Purkinje cell loss and astrocytic reaction in the cerebellum after traumatic brain injury. Neurosci Lett 481:178\u2013182","journal-title":"Neurosci Lett"},{"key":"10_CR255","doi-asserted-by":"publisher","first-page":"4697","DOI":"10.1073\/pnas.1300398110","volume":"110","author":"A Serio","year":"2013","unstructured":"Serio A, Bilican B, Barmada SJ, Ando DM, Zhao C, Siller R, Burr K, Haghi G, Story D, Nishimura AL, Carrasco MA, Phatnani HP, Shum C, Wilmut I, Maniatis T, Shaw CE, Finkbeiner S, Chandran S (2013) Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy. Proc Natl Acad Sci USA 110:4697\u20134702","journal-title":"Proc Natl Acad Sci USA"},{"key":"10_CR256","doi-asserted-by":"publisher","first-page":"2534","DOI":"10.1002\/1873-3468.12765","volume":"591","author":"J Sheinberger","year":"2017","unstructured":"Sheinberger J, Shav-Tal Y (2017) mRNPs meet stress granules. FEBS Lett 591:2534\u20132542","journal-title":"FEBS Lett"},{"key":"10_CR257","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1016\/S0006-8993(01)02926-2","volume":"917","author":"N Shibata","year":"2001","unstructured":"Shibata N, Nagai R, Uchida K, Horiuchi S, Yamada S, Hirano A, Kawaguchi M, Yamamoto T, Sasaki S, Kobayashi M (2001) Morphological evidence for lipid peroxidation and protein glycoxidation in spinal cords from sporadic amyotrophic lateral sclerosis patients. Brain Res 917:97\u2013104","journal-title":"Brain Res"},{"key":"10_CR258","doi-asserted-by":"publisher","first-page":"170","DOI":"10.1016\/j.brainres.2004.05.110","volume":"1019","author":"N Shibata","year":"2004","unstructured":"Shibata N, Yamada S, Uchida K, Hirano A, Sakoda S, Fujimura H, Sasaki S, Iwata M, Toi S, Kawaguchi M, Yamamoto T, Kobayashi M (2004) Accumulation of protein-bound 4-hydroxy-2-hexenal in spinal cords from patients with sporadic amyotrophic lateral sclerosis. Brain Res 1019:170\u2013177","journal-title":"Brain Res"},{"key":"10_CR259","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1111\/j.1440-1789.2006.00746.x","volume":"27","author":"N Shibata","year":"2007","unstructured":"Shibata N et al (2007) Protein-bound crotonaldehyde accumulates in the spinal cord of superoxide dismutase-1 mutation-associated familial amyotrophic lateral sclerosis and its transgenic mouse model. Neuropathol: Off J Jpn Soc Neuropathol 27:49\u201361","journal-title":"Neuropathol: Off J Jpn Soc Neuropathol"},{"key":"10_CR260","doi-asserted-by":"publisher","first-page":"520","DOI":"10.1038\/gt.2017.34","volume":"24","author":"NN Singh","year":"2017","unstructured":"Singh NN, Howell MD, Androphy EJ, Singh RN (2017) How the discovery of ISS-N1 led to the first medical therapy for spinal muscular atrophy. Gene Ther 24:520\u2013526","journal-title":"Gene Ther"},{"key":"10_CR261","first-page":"299","volume":"1860","author":"RN Singh","year":"2017","unstructured":"Singh RN, Howell MD, Ottesen EW, Singh NN (2017) Diverse role of survival motor neuron protein. Biochem Biophys Acta 1860:299\u2013315","journal-title":"Biochem Biophys Acta"},{"key":"10_CR262","doi-asserted-by":"publisher","first-page":"3409","DOI":"10.1093\/hmg\/ddx230","volume":"26","author":"SL Sison","year":"2017","unstructured":"Sison SL, Patitucci TN, Seminary ER, Villalon E, Lorson CL, Ebert AD (2017) Astrocyte-produced miR-146a as a mediator of motor neuron loss in spinal muscular atrophy. Hum Mol Genet 26:3409\u20133420","journal-title":"Hum Mol Genet"},{"key":"10_CR263","doi-asserted-by":"publisher","first-page":"274","DOI":"10.1016\/j.jprot.2013.07.028","volume":"91","author":"A Skorupa","year":"2013","unstructured":"Skorupa A, Urbach S, Vigy O, King MA, Chaumont-Dubel S, Prehn JH, Marin P (2013) Angiogenin induces modifications in the astrocyte secretome: relevance to amyotrophic lateral sclerosis. J Proteomics 91:274\u2013285","journal-title":"J Proteomics"},{"key":"10_CR264","doi-asserted-by":"publisher","first-page":"5024","DOI":"10.1523\/JNEUROSCI.6366-11.2012","volume":"32","author":"A Skorupa","year":"2012","unstructured":"Skorupa A, King MA, Aparicio IM, Dussmann H, Coughlan K, Breen B, Kieran D, Concannon CG, Marin P, Prehn JH (2012) Motoneurons secrete angiogenin to induce RNA cleavage in astroglia. J Neurosci: Off J Soc Neurosci 32:5024\u20135038","journal-title":"J Neurosci: Off J Soc Neurosci"},{"key":"10_CR265","unstructured":"Smith BN et al (2017) Mutations in the vesicular trafficking protein annexin A11 are associated with amyotrophic lateral sclerosis. Sci Transl Med 9"},{"key":"10_CR266","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1016\/0092-8674(91)90568-J","volume":"67","author":"B Sommer","year":"1991","unstructured":"Sommer B, Kohler M, Sprengel R, Seeburg PH (1991) RNA editing in brain controls a determinant of ion flow in glutamate-gated channels. Cell 67:11\u201319","journal-title":"Cell"},{"key":"10_CR267","doi-asserted-by":"publisher","first-page":"397","DOI":"10.1038\/nm.4052","volume":"22","author":"S Song","year":"2016","unstructured":"Song S, Miranda CJ, Braun L, Meyer K, Frakes AE, Ferraiuolo L, Likhite S, Bevan AK, Foust KD, McConnell MJ, Walker CM, Kaspar BK (2016) Major histocompatibility complex class I molecules protect motor neurons from astrocyte-induced toxicity in amyotrophic lateral sclerosis. Nat Med 22:397\u2013403","journal-title":"Nat Med"},{"key":"10_CR268","doi-asserted-by":"publisher","first-page":"1668","DOI":"10.1126\/science.1154584","volume":"319","author":"J Sreedharan","year":"2008","unstructured":"Sreedharan J, Blair IP, Tripathi VB, Hu X, Vance C, Rogelj B, Ackerley S, Durnall JC, Williams KL, Buratti E, Baralle F, de Belleroche J, Mitchell JD, Leigh PN, Al-Chalabi A, Miller CC, Nicholson G, Shaw CE (2008) TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science 319:1668\u20131672","journal-title":"Science"},{"key":"10_CR269","doi-asserted-by":"publisher","first-page":"258","DOI":"10.1016\/j.freeradbiomed.2017.03.034","volume":"108","author":"S Stamenkovic","year":"2017","unstructured":"Stamenkovic S, Pavicevic A, Mojovic M, Popovic-Bijelic A, Selakovic V, Andjus P, Bacic G (2017) In vivo EPR pharmacokinetic evaluation of the redox status and the blood brain barrier permeability in the SOD1(G93A) ALS rat model. Free Radic Biol Med 108:258\u2013269","journal-title":"Free Radic Biol Med"},{"key":"10_CR270","doi-asserted-by":"publisher","first-page":"114","DOI":"10.1016\/S0022-510X(00)00351-8","volume":"177","author":"A Stieber","year":"2000","unstructured":"Stieber A, Gonatas JO, Gonatas NK (2000) Aggregates of mutant protein appear progressively in dendrites, in periaxonal processes of oligodendrocytes, and in neuronal and astrocytic perikarya of mice expressing the SOD1(G93A) mutation of familial amyotrophic lateral sclerosis. J Neurol Sci 177:114\u2013123","journal-title":"J Neurol Sci"},{"key":"10_CR271","doi-asserted-by":"publisher","first-page":"E6993","DOI":"10.1073\/pnas.1520639112","volume":"112","author":"S Sun","year":"2015","unstructured":"Sun S, Sun Y, Ling SC, Ferraiuolo L, McAlonis-Downes M, Zou Y, Drenner K, Wang Y, Ditsworth D, Tokunaga S, Kopelevich A, Kaspar BK, Lagier-Tourenne C, Cleveland DW (2015) Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS. Proc Natl Acad Sci USA 112:E6993\u20137002","journal-title":"Proc Natl Acad Sci USA"},{"key":"10_CR272","doi-asserted-by":"publisher","first-page":"2429","DOI":"10.1084\/jem.20111313","volume":"208","author":"V Swarup","year":"2011","unstructured":"Swarup V, Phaneuf D, Dupre N, Petri S, Strong M, Kriz J, Julien JP (2011) Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor kappaB-mediated pathogenic pathways. J Exp Med 208:2429\u20132447","journal-title":"J Exp Med"},{"key":"10_CR273","doi-asserted-by":"publisher","first-page":"3489","DOI":"10.1007\/s00018-015-1937-8","volume":"72","author":"K Takahashi","year":"2015","unstructured":"Takahashi K, Foster JB, Lin CL (2015) Glutamate transporter EAAT2: regulation, function, and potential as a therapeutic target for neurological and psychiatric disease. Cell Mol Life Sci: CMLS 72:3489\u20133506","journal-title":"Cell Mol Life Sci: CMLS"},{"key":"10_CR274","doi-asserted-by":"publisher","first-page":"529","DOI":"10.1038\/gt.2017.52","volume":"24","author":"K Talbot","year":"2017","unstructured":"Talbot K, Tizzano EF (2017) The clinical landscape for SMA in a new therapeutic era. Gene Ther 24:529\u2013533","journal-title":"Gene Ther"},{"key":"10_CR275","doi-asserted-by":"publisher","first-page":"197","DOI":"10.1038\/nature20413","volume":"539","author":"JP Taylor","year":"2016","unstructured":"Taylor JP, Brown RH Jr, Cleveland DW (2016) Decoding ALS: from genes to mechanism. Nature 539:197\u2013206","journal-title":"Nature"},{"key":"10_CR276","doi-asserted-by":"publisher","first-page":"96","DOI":"10.1111\/jnc.14309","volume":"145","author":"EA Thomas","year":"2018","unstructured":"Thomas EA, D\u2019Mello SR (2018) Complex neuroprotective and neurotoxic effects of histone deacetylases. J Neurochem 145:96\u2013110","journal-title":"J Neurochem"},{"key":"10_CR277","doi-asserted-by":"crossref","unstructured":"Thomsen GM, Avalos P, Ma AA, Alkaslasi M, Cho N, Wyss L, Vit JP, Godoy M, Suezaki P, Shelest O, Bankiewicz KS, Svendsen CN (2018) Transplantation of neural progenitor cells expressing glial cell line-derived neurotrophic factor into the motor cortex as a strategy to treat amyotrophic lateral sclerosis. Stem Cells","DOI":"10.1002\/stem.2825"},{"key":"10_CR278","doi-asserted-by":"crossref","unstructured":"Tian YP, Che FY, Su QP, Lu YC, You CP, Huang LM, Wang SG, Wang L, Yu JX (2017) Effects of mutant TDP-43 on the Nrf2\/ARE pathway and protein expression of MafK and JDP2 in NSC-34 cells. Genet Mol Res: GMR 16","DOI":"10.4238\/gmr16029638"},{"key":"10_CR279","doi-asserted-by":"crossref","unstructured":"Tong J, Huang C, Bi F, Wu Q, Huang B, Liu X, Li F, Zhou H, Xia XG (2013) Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats. EMBO J","DOI":"10.1038\/emboj.2013.122"},{"key":"10_CR280","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1111\/jnc.13154","volume":"135","author":"M Tortarolo","year":"2015","unstructured":"Tortarolo M, Vallarola A, Lidonnici D, Battaglia E, Gensano F, Spaltro G, Fiordaliso F, Corbelli A, Garetto S, Martini E, Pasetto L, Kallikourdis M, Bonetto V, Bendotti C (2015) Lack of TNF-alpha receptor type 2 protects motor neurons in a cellular model of amyotrophic lateral sclerosis and in mutant SOD1 mice but does not affect disease progression. J Neurochem 135:109\u2013124","journal-title":"J Neurochem"},{"key":"10_CR281","doi-asserted-by":"publisher","first-page":"667","DOI":"10.1016\/j.stemcr.2017.06.008","volume":"9","author":"P Tripathi","year":"2017","unstructured":"Tripathi P, Rodriguez-Muela N, Klim JR, de Boer AS, Agrawal S, Sandoe J, Lopes CS, Ogliari KS, Williams LA, Shear M, Rubin LL, Eggan K, Zhou Q (2017) Reactive astrocytes promote ALS-like degeneration and intracellular protein aggregation in human motor neurons by disrupting autophagy through TGF-beta1. Stem Cell Rep 9:667\u2013680","journal-title":"Stem Cell Rep"},{"key":"10_CR282","doi-asserted-by":"publisher","first-page":"2224","DOI":"10.1007\/s00415-017-8619-4","volume":"264","author":"F Trojsi","year":"2017","unstructured":"Trojsi F et al (2017) Comorbidity of dementia with amyotrophic lateral sclerosis (ALS): insights from a large multicenter Italian cohort. J Neurol 264:2224\u20132231","journal-title":"J Neurol"},{"key":"10_CR283","doi-asserted-by":"publisher","first-page":"131","DOI":"10.3389\/fnmol.2018.00131","volume":"11","author":"A Trostchansky","year":"2018","unstructured":"Trostchansky A, Mastrogiovanni M, Miquel E, Rodriguez-Bottero S, Martinez-Palma L, Cassina P, Rubbo H (2018) Profile of arachidonic acid-derived inflammatory markers and its modulation by nitro-oleic acid in an inherited model of amyotrophic lateral sclerosis. Front Mol Neurosci 11:131","journal-title":"Front Mol Neurosci"},{"key":"10_CR284","doi-asserted-by":"publisher","first-page":"2207","DOI":"10.1111\/j.1460-9568.1997.tb01388.x","volume":"9","author":"D Trotti","year":"1997","unstructured":"Trotti D, Nussberger S, Volterra A, Hediger MA (1997) Differential modulation of the uptake currents by redox interconversion of cysteine residues in the human neuronal glutamate transporter EAAC1. Eur J Neurosci 9:2207\u20132212","journal-title":"Eur J Neurosci"},{"key":"10_CR285","doi-asserted-by":"publisher","first-page":"427","DOI":"10.1038\/8091","volume":"2","author":"D Trotti","year":"1999","unstructured":"Trotti D, Rolfs A, Danbolt NC, Brown RH Jr, Hediger MA (1999) SOD1 mutants linked to amyotrophic lateral sclerosis selectively inactivate a glial glutamate transporter. Nat Neurosci 2:427\u2013433","journal-title":"Nat Neurosci"},{"key":"10_CR286","doi-asserted-by":"publisher","first-page":"5976","DOI":"10.1074\/jbc.271.11.5976","volume":"271","author":"D Trotti","year":"1996","unstructured":"Trotti D, Rossi D, Gjesdal O, Levy LM, Racagni G, Danbolt NC, Volterra A (1996) Peroxynitrite inhibits glutamate transporter subtypes. J Biol Chem 271:5976\u20135979","journal-title":"J Biol Chem"},{"key":"10_CR287","doi-asserted-by":"publisher","first-page":"1164","DOI":"10.1038\/s41467-017-01283-z","volume":"8","author":"GE Tyzack","year":"2017","unstructured":"Tyzack GE, Hall CE, Sibley CR, Cymes T, Forostyak S, Carlino G, Meyer IF, Schiavo G, Zhang SC, Gibbons GM, Newcombe J, Patani R, Lakatos A (2017) A neuroprotective astrocyte state is induced by neuronal signal EphB1 but fails in ALS models. Nat Commun 8:1164","journal-title":"Nat Commun"},{"key":"10_CR288","doi-asserted-by":"publisher","first-page":"13395","DOI":"10.1074\/jbc.M011176200","volume":"276","author":"H Uranishi","year":"2001","unstructured":"Uranishi H, Tetsuka T, Yamashita M, Asamitsu K, Shimizu M, Itoh M, Okamoto T (2001) Involvement of the pro-oncoprotein TLS (translocated in liposarcoma) in nuclear factor-kappa B p65-mediated transcription as a coactivator. J Biol Chem 276:13395\u201313401","journal-title":"J Biol Chem"},{"key":"10_CR289","doi-asserted-by":"crossref","unstructured":"Valori CF, Ning K, Wyles M, Mead RJ, Grierson AJ, Shaw PJ, Azzouz M (2010) Systemic delivery of scAAV9 expressing SMN prolongs survival in a model of spinal muscular atrophy. Sci Transl Med 2:35ra42","DOI":"10.1126\/scitranslmed.3000830"},{"key":"10_CR290","doi-asserted-by":"publisher","first-page":"14825","DOI":"10.1073\/pnas.0705046104","volume":"104","author":"P Damme Van","year":"2007","unstructured":"Van Damme P, Bogaert E, Dewil M, Hersmus N, Kiraly D, Scheveneels W, Bockx I, Braeken D, Verpoorten N, Verhoeven K, Timmerman V, Herijgers P, Callewaert G, Carmeliet P, Van Den Bosch L, Robberecht W (2007) Astrocytes regulate GluR2 expression in motor neurons and their vulnerability to excitotoxicity. Proc Natl Acad Sci USA 104:14825\u201314830","journal-title":"Proc Natl Acad Sci USA"},{"key":"10_CR291","doi-asserted-by":"publisher","first-page":"2084","DOI":"10.1016\/S0140-6736(17)31287-4","volume":"390","author":"MA Es van","year":"2017","unstructured":"van Es MA, Hardiman O, Chio A, Al-Chalabi A, Pasterkamp RJ, Veldink JH, van den Berg LH (2017) Amyotrophic lateral sclerosis. Lancet 390:2084\u20132098","journal-title":"Lancet"},{"key":"10_CR292","doi-asserted-by":"publisher","first-page":"1208","DOI":"10.1126\/science.1165942","volume":"323","author":"C Vance","year":"2009","unstructured":"Vance C et al (2009) Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Science 323:1208\u20131211","journal-title":"Science"},{"key":"10_CR293","doi-asserted-by":"publisher","first-page":"687","DOI":"10.1111\/j.1471-4159.2006.03742.x","volume":"97","author":"MR Vargas","year":"2006","unstructured":"Vargas MR, Pehar M, Cassina P, Beckman JS, Barbeito L (2006) Increased glutathione biosynthesis by Nrf2 activation in astrocytes prevents p75NTR-dependent motor neuron apoptosis. J Neurochem 97:687\u2013696","journal-title":"J Neurochem"},{"key":"10_CR294","doi-asserted-by":"publisher","first-page":"13574","DOI":"10.1523\/JNEUROSCI.4099-08.2008","volume":"28","author":"MR Vargas","year":"2008","unstructured":"Vargas MR, Johnson DA, Sirkis DW, Messing A, Johnson JA (2008) Nrf2 activation in astrocytes protects against neurodegeneration in mouse models of familial amyotrophic lateral sclerosis. J Neurosci: Off J Soc Neurosci 28:13574\u201313581","journal-title":"J Neurosci: Off J Soc Neurosci"},{"key":"10_CR295","doi-asserted-by":"publisher","first-page":"25571","DOI":"10.1074\/jbc.M501920200","volume":"280","author":"MR Vargas","year":"2005","unstructured":"Vargas MR, Pehar M, Cassina P, Martinez-Palma L, Thompson JA, Beckman JS, Barbeito L (2005) Fibroblast growth factor-1 induces heme oxygenase-1 via nuclear factor erythroid 2-related factor 2 (Nrf2) in spinal cord astrocytes: consequences for motor neuron survival. J Biol Chem 280:25571\u201325579","journal-title":"J Biol Chem"},{"key":"10_CR296","doi-asserted-by":"publisher","first-page":"e56625","DOI":"10.1371\/journal.pone.0056625","volume":"8","author":"MR Vargas","year":"2013","unstructured":"Vargas MR, Burton NC, Gan L, Johnson DA, Schafer M, Werner S, Johnson JA (2013) Absence of Nrf2 or Its selective overexpression in neurons and muscle does not affect survival in ALS-linked mutant hSOD1 mouse models. PLoS ONE 8:e56625","journal-title":"PLoS ONE"},{"key":"10_CR297","doi-asserted-by":"publisher","first-page":"749","DOI":"10.1002\/glia.23279","volume":"66","author":"M Vergouts","year":"2018","unstructured":"Vergouts M, Doyen PJ, Peeters M, Opsomer R, Hermans E (2018) Constitutive downregulation protein kinase C epsilon in hSOD1(G93A) astrocytes influences mGluR5 signaling and the regulation of glutamate uptake. Glia 66:749\u2013761","journal-title":"Glia"},{"key":"10_CR298","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1186\/s13023-017-0671-8","volume":"12","author":"IEC Verhaart","year":"2017","unstructured":"Verhaart IEC, Robertson A, Wilson IJ, Aartsma-Rus A, Cameron S, Jones CC, Cook SF, Lochmuller H (2017) Prevalence, incidence and carrier frequency of 5q-linked spinal muscular atrophy - a literature review. Orphanet J Rare Dis 12:124","journal-title":"Orphanet J Rare Dis"},{"key":"10_CR299","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1016\/j.neubiorev.2018.03.028","volume":"90","author":"ICM Verheggen","year":"2018","unstructured":"Verheggen ICM, Van Boxtel MPJ, Verhey FRJ, Jansen JFA, Backes WH (2018) Interaction between blood-brain barrier and glymphatic system in solute clearance. Neurosci Biobehav Rev 90:26\u201333","journal-title":"Neurosci Biobehav Rev"},{"key":"10_CR300","doi-asserted-by":"publisher","first-page":"239","DOI":"10.15252\/embj.201592705","volume":"35","author":"A Verkhratsky","year":"2016","unstructured":"Verkhratsky A, Matteoli M, Parpura V, Mothet JP, Zorec R (2016) Astrocytes as secretory cells of the central nervous system: idiosyncrasies of vesicular secretion. EMBO J 35:239\u2013257","journal-title":"EMBO J"},{"key":"10_CR301","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1007\/978-3-319-40764-7_1","volume":"949","author":"R Bernhardi von","year":"2016","unstructured":"von Bernhardi R, Eugenin-von Bernhardi J, Flores B, Eugenin Leon J (2016) Glial Cells and Integrity of the Nervous System. Adv Exp Med Biol 949:1\u201324","journal-title":"Adv Exp Med Biol"},{"key":"10_CR302","doi-asserted-by":"publisher","first-page":"2453","DOI":"10.3892\/mmr.2014.2087","volume":"9","author":"F Wang","year":"2014","unstructured":"Wang F, Lu Y, Qi F, Su Q, Wang L, You C, Che F, Yu J (2014) Effect of the human SOD1-G93A gene on the Nrf2\/ARE signaling pathway in NSC-34 cells. Mol Med Rep 9:2453\u20132458","journal-title":"Mol Med Rep"},{"key":"10_CR303","doi-asserted-by":"publisher","first-page":"286","DOI":"10.1093\/hmg\/ddq463","volume":"20","author":"L Wang","year":"2011","unstructured":"Wang L, Gutmann DH, Roos RP (2011) Astrocyte loss of mutant SOD1 delays ALS disease onset and progression in G85R transgenic mice. Hum Mol Genet 20:286\u2013293","journal-title":"Hum Mol Genet"},{"key":"10_CR304","doi-asserted-by":"publisher","first-page":"986","DOI":"10.1007\/s11064-016-2131-6","volume":"42","author":"W Wang","year":"2017","unstructured":"Wang W, Duan W, Wang Y, Wen D, Liu Y, Li Z, Hu H, Cui H, Cui C, Lin H, Li C (2017) Intrathecal delivery of ssAAV9-DAO extends survival in SOD1(G93A) ALS mice. Neurochem Res 42:986\u2013996","journal-title":"Neurochem Res"},{"key":"10_CR305","doi-asserted-by":"crossref","unstructured":"Watanabe-Matsumoto S, Moriwaki Y, Okuda T, Ohara S, Yamanaka K, Abe Y, Yasui M, Misawa H (2017) Dissociation of blood-brain barrier disruption and disease manifestation in an aquaporin-4-deficient mouse model of amyotrophic lateral sclerosis. Neurosci Res","DOI":"10.1016\/j.neures.2017.11.001"},{"key":"10_CR306","doi-asserted-by":"publisher","first-page":"111","DOI":"10.1007\/s00401-012-1039-8","volume":"125","author":"EA Winkler","year":"2013","unstructured":"Winkler EA, Sengillo JD, Sullivan JS, Henkel JS, Appel SH, Zlokovic BV (2013) Blood-spinal cord barrier breakdown and pericyte reductions in amyotrophic lateral sclerosis. Acta Neuropathol 125:111\u2013120","journal-title":"Acta Neuropathol"},{"key":"10_CR307","doi-asserted-by":"crossref","unstructured":"Wirth B, Mendoza Ferreira N, Torres-Benito L (2017) Spinal Muscular Atrophy Disease Modifiers","DOI":"10.1016\/B978-0-12-803685-3.00012-4"},{"key":"10_CR308","doi-asserted-by":"publisher","first-page":"294","DOI":"10.1097\/00019052-199508000-00008","volume":"8","author":"PC Wong","year":"1995","unstructured":"Wong PC, Borchelt DR (1995) Motor neuron disease caused by mutations in superoxide dismutase 1. Curr Opin Neurol 8:294\u2013301","journal-title":"Curr Opin Neurol"},{"key":"10_CR309","doi-asserted-by":"publisher","first-page":"499","DOI":"10.1038\/nature11280","volume":"488","author":"CH Wu","year":"2012","unstructured":"Wu CH et al (2012) Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis. Nature 488:499\u2013503","journal-title":"Nature"},{"key":"10_CR310","doi-asserted-by":"publisher","first-page":"251","DOI":"10.1038\/nn2047","volume":"11","author":"K Yamanaka","year":"2008","unstructured":"Yamanaka K, Chun SJ, Boillee S, Fujimori-Tonou N, Yamashita H, Gutmann DH, Takahashi R, Misawa H, Cleveland DW (2008) Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis. Nat Neurosci 11:251\u2013253","journal-title":"Nat Neurosci"},{"key":"10_CR311","doi-asserted-by":"publisher","first-page":"E1121","DOI":"10.1073\/pnas.1322641111","volume":"111","author":"C Yang","year":"2014","unstructured":"Yang C, Wang H, Qiao T, Yang B, Aliaga L, Qiu L, Tan W, Salameh J, McKenna-Yasek DM, Smith T, Peng L, Moore MJ, Brown RH Jr, Cai H, Xu Z (2014) Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis. Proc Natl Acad Sci USA 111:E1121\u20131129","journal-title":"Proc Natl Acad Sci USA"},{"key":"10_CR312","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1016\/j.ejps.2018.04.026","volume":"120","author":"T Yang","year":"2018","unstructured":"Yang T, Ferrill L, Gallant L, McGillicuddy S, Fernandes T, Schields N, Bai S (2018) Verapamil and riluzole cocktail liposomes overcome pharmacoresistance by inhibiting P-glycoprotein in brain endothelial and astrocyte cells: A potent approach to treat amyotrophic lateral sclerosis. Eur J Pharm Sci: Off J Eur Fed Pharm Sci 120:30\u201339","journal-title":"Eur J Pharm Sci: Off J Eur Fed Pharm Sci"},{"key":"10_CR313","doi-asserted-by":"publisher","first-page":"880","DOI":"10.1016\/j.neuron.2009.02.010","volume":"61","author":"Y Yang","year":"2009","unstructured":"Yang Y, Gozen O, Watkins A, Lorenzini I, Lepore A, Gao Y, Vidensky S, Brennan J, Poulsen D, Won Park J, Li Jeon N, Robinson MB, Rothstein JD (2009) Presynaptic regulation of astroglial excitatory neurotransmitter transporter GLT1. Neuron 61:880\u2013894","journal-title":"Neuron"},{"key":"10_CR314","doi-asserted-by":"publisher","first-page":"160","DOI":"10.1038\/ng1001-160","volume":"29","author":"Y Yang","year":"2001","unstructured":"Yang Y, Hentati A, Deng HX, Dabbagh O, Sasaki T, Hirano M, Hung WY, Ouahchi K, Yan J, Azim AC, Cole N, Gascon G, Yagmour A, Ben-Hamida M, Pericak-Vance M, Hentati F, Siddique T (2001) The gene encoding alsin, a protein with three guanine-nucleotide exchange factor domains, is mutated in a form of recessive amyotrophic lateral sclerosis. Nat Genet 29:160\u2013165","journal-title":"Nat Genet"},{"key":"10_CR315","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.mcn.2018.05.004","volume":"90","author":"X Yin","year":"2018","unstructured":"Yin X, Wang S, Qi Y, Wang X, Jiang H, Wang T, Yang Y, Wang Y, Zhang C, Feng H (2018) Astrocyte elevated gene-1 is a novel regulator of astrogliosis and excitatory amino acid transporter-2 via interplaying with nuclear factor-kappaB signaling in astrocytes from amyotrophic lateral sclerosis mouse model with hSOD1(G93A) mutation. Mol Cell Neurosci 90:1\u201311","journal-title":"Mol Cell Neurosci"},{"key":"10_CR316","doi-asserted-by":"publisher","first-page":"6391","DOI":"10.1523\/JNEUROSCI.6221-11.2012","volume":"32","author":"JL Zamanian","year":"2012","unstructured":"Zamanian JL, Xu L, Foo LC, Nouri N, Zhou L, Giffard RG, Barres BA (2012) Genomic analysis of reactive astrogliosis. J Neurosci: Off J Soc Neurosci 32:6391\u20136410","journal-title":"J Neurosci: Off J Soc Neurosci"},{"key":"10_CR317","doi-asserted-by":"publisher","first-page":"1138","DOI":"10.1126\/science.aaa1934","volume":"347","author":"A Zeisel","year":"2015","unstructured":"Zeisel A, Munoz-Manchado AB, Codeluppi S, Lonnerberg P, La Manno G, Jureus A, Marques S, Munguba H, He L, Betsholtz C, Rolny C, Castelo-Branco G, Hjerling-Leffler J, Linnarsson S (2015) Brain structure. Cell types in the mouse cortex and hippocampus revealed by single-cell RNA-seq. Science 347:1138\u20131142","journal-title":"Science"},{"key":"10_CR318","doi-asserted-by":"publisher","first-page":"420","DOI":"10.1038\/nn2073","volume":"11","author":"Z Zhong","year":"2008","unstructured":"Zhong Z, Deane R, Ali Z, Parisi M, Shapovalov Y, O\u2019Banion MK, Stojanovic K, Sagare A, Boillee S, Cleveland DW, Zlokovic BV (2008) ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration. Nat Neurosci 11:420\u2013422","journal-title":"Nat Neurosci"},{"key":"10_CR319","doi-asserted-by":"publisher","first-page":"2543","DOI":"10.1523\/JNEUROSCI.3534-15.2016","volume":"36","author":"C Zhou","year":"2016","unstructured":"Zhou C, Feng Z, Ko CP (2016) Defects in Motoneuron-Astrocyte Interactions in Spinal Muscular Atrophy. The Journal of neuroscience: the official journal of the Society for Neuroscience 36:2543\u20132553","journal-title":"The Journal of neuroscience: the official journal of the Society for Neuroscience"},{"key":"10_CR320","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1111\/jnc.14229","volume":"145","author":"F Zhou","year":"2018","unstructured":"Zhou F, Zhang C, Guan Y, Chen Y, Lu Q, Jie L, Gao H, Du H, Zhang H, Liu Y, Wang X (2018) Screening the expression characteristics of several miRNAs in G93A-SOD1 transgenic mouse: altered expression of miRNA-124 is associated with astrocyte differentiation by targeting Sox2 and Sox9. J Neurochem 145:51\u201367","journal-title":"J Neurochem"},{"key":"10_CR321","doi-asserted-by":"publisher","first-page":"409","DOI":"10.1016\/j.nicl.2015.01.009","volume":"7","author":"NR Zurcher","year":"2015","unstructured":"Zurcher NR, Loggia ML, Lawson R, Chonde DB, Izquierdo-Garcia D, Yasek JE, Akeju O, Catana C, Rosen BR, Cudkowicz ME, Hooker JM, Atassi N (2015) Increased in vivo glial activation in patients with amyotrophic lateral sclerosis: assessed with [(11)C]-PBR28. NeuroImage Clinical 7:409\u2013414","journal-title":"NeuroImage Clinical"}],"container-title":["Advances in Experimental Medicine and Biology","Neuroglia in Neurodegenerative Diseases"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/978-981-13-9913-8_10","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,7,24]],"date-time":"2024-07-24T13:04:15Z","timestamp":1721826255000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/978-981-13-9913-8_10"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019]]},"ISBN":["9789811399121","9789811399138"],"references-count":321,"URL":"https:\/\/doi.org\/10.1007\/978-981-13-9913-8_10","relation":{},"ISSN":["0065-2598","2214-8019"],"issn-type":[{"value":"0065-2598","type":"print"},{"value":"2214-8019","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019]]},"assertion":[{"value":"4 October 2019","order":1,"name":"first_online","label":"First Online","group":{"name":"ChapterHistory","label":"Chapter History"}}]}}