{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T18:53:51Z","timestamp":1791053631402,"version":"4.1.0"},"reference-count":224,"publisher":"Springer Science and Business Media LLC","issue":"5-6","license":[{"start":{"date-parts":[[2013,3,29]],"date-time":"2013-03-29T00:00:00Z","timestamp":1364515200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/2.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Mol Genet Genomics"],"published-print":{"date-parts":[[2013,6]]},"DOI":"10.1007\/s00438-013-0741-0","type":"journal-article","created":{"date-parts":[[2013,3,28]],"date-time":"2013-03-28T15:08:05Z","timestamp":1364483285000},"page":"207-229","source":"Crossref","is-referenced-by-count":101,"title":["The wobbler mouse, an ALS animal model"],"prefix":"10.1007","volume":"288","author":[{"given":"Jakob Maximilian","family":"Moser","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Bigini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Schmitt-John","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2013,3,29]]},"reference":[{"key":"741_CR1","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1002\/(SICI)1097-4547(19970401)48:1<63::AID-JNR6>3.0.CO;2-C","volume":"48","author":"K Abe","year":"1997","unstructured":"Abe K, Morita S, Kikuchi T, Itoyama Y (1997) Protective effect of a novel free radical scavenger, OPC-14117, on wobbler mouse motor neuron disease. J Neurosci Res 48:63\u201370","journal-title":"J Neurosci Res"},{"key":"741_CR2","first-page":"806306","volume":"2012","author":"EY Achi","year":"2012","unstructured":"Achi EY, Rudnicki SA (2012) ALS and frontotemporal dysfunction: a review. Neurol Res Int 2012:806306","journal-title":"Neurol Res Int"},{"key":"741_CR3","doi-asserted-by":"publisher","first-page":"165","DOI":"10.1083\/jcb.150.1.165","volume":"150","author":"S Ackerley","year":"2000","unstructured":"Ackerley S, Grierson AJ, Brownlees J, Thornhill P, Anderton BH et al (2000) Glutamate slows axonal transport of neurofilaments in transfected neurons. J Cell Biol 150:165\u2013176","journal-title":"J Cell Biol"},{"key":"741_CR4","doi-asserted-by":"publisher","first-page":"191","DOI":"10.1016\/S0014-5793(04)00179-6","volume":"561","author":"O Agbulut","year":"2004","unstructured":"Agbulut O, Noirez P, Butler-Browne G, Jockusch H (2004) Specific isomyosin proportions in hyperexcitable and physiologically denervated mouse muscle. FEBS Lett 561:191\u2013194","journal-title":"FEBS Lett"},{"key":"741_CR5","doi-asserted-by":"publisher","first-page":"913","DOI":"10.1002\/ana.22534","volume":"70","author":"A Al-Saif","year":"2011","unstructured":"Al-Saif A, Al-Mohanna F, Bohlega S (2011) A mutation in sigma-1 receptor causes juvenile amyotrophic lateral sclerosis. Ann Neurol 70:913\u2013919","journal-title":"Ann Neurol"},{"key":"741_CR6","first-page":"63","volume":"76","author":"JM Andrews","year":"1974","unstructured":"Andrews JM, Gardner MB, Wolfgram FJ, Ellison GW, Porter DD, Brandkamp WW (1974) Studies on a murine form of spontaneous lower motor neuron degeneration\u2014the wobbler (wa) mouse. Am J Pathol 76:63\u201378","journal-title":"Am J Pathol"},{"key":"741_CR7","doi-asserted-by":"publisher","first-page":"30152","DOI":"10.1074\/jbc.M603393200","volume":"281","author":"JD Atkin","year":"2006","unstructured":"Atkin JD, Farg MA, Turner BJ, Tomas D, Lysaght JA et al (2006) Induction of the unfolded protein response in familial amyotrophic lateral sclerosis and association of protein-disulfide isomerase with superoxide dismutase 1. J Biol Chem 281:30152\u201330165","journal-title":"J Biol Chem"},{"key":"741_CR8","doi-asserted-by":"publisher","first-page":"400","DOI":"10.1016\/j.nbd.2008.02.009","volume":"30","author":"JD Atkin","year":"2008","unstructured":"Atkin JD, Farg MA, Walker AK, McLean C, Tomas D, Horne MK (2008) Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis. Neurobiol Dis 30:400\u2013407","journal-title":"Neurobiol Dis"},{"key":"741_CR9","doi-asserted-by":"publisher","first-page":"286","DOI":"10.1002\/(SICI)1097-0177(199707)209:3<286::AID-AJA4>3.0.CO;2-E","volume":"209","author":"M Augustin","year":"1997","unstructured":"Augustin M, Heimann P, Rathke S, Jockusch H (1997) Spinal muscular atrophy gene wobbler of the mouse: evidence from chimeric spinal cord and testis for cell-autonomous function. Dev Dyn 209:286\u2013295","journal-title":"Dev Dyn"},{"key":"741_CR10","doi-asserted-by":"publisher","first-page":"5229","DOI":"10.1021\/pr900569d","volume":"8","author":"A Bastone","year":"2009","unstructured":"Bastone A, Fumagalli E, Bigini P, Perini P, Bernardinello D et al (2009) Proteomic profiling of cervical and lumbar spinal cord reveals potential protective mechanisms in the wobbler mouse, a model of motor neuron degeneration. J Proteome Res 8:5229\u20135240","journal-title":"J Proteome Res"},{"key":"741_CR11","doi-asserted-by":"publisher","first-page":"531","DOI":"10.1083\/jcb.147.3.531","volume":"147","author":"JM Beaulieu","year":"1999","unstructured":"Beaulieu JM, Nguyen MD, Julien JP (1999) Late onset of motor neurons in mice overexpressing wild-type peripherin. J Cell Biol 147:531\u2013544","journal-title":"J Cell Biol"},{"key":"741_CR12","doi-asserted-by":"publisher","first-page":"100","DOI":"10.1080\/14660820510028412","volume":"6","author":"M Beck","year":"2005","unstructured":"Beck M, Flachenecker P, Magnus T, Giess R, Reiners K et al (2005) Autonomic dysfunction in ALS: a preliminary study on the effects of intrathecal BDNF. Amyotroph Lateral Scler Other Motor Neuron Disord 6:100\u2013103","journal-title":"Amyotroph Lateral Scler Other Motor Neuron Disord"},{"key":"741_CR13","doi-asserted-by":"publisher","first-page":"15558","DOI":"10.1073\/pnas.0807419105","volume":"105","author":"DR Beers","year":"2008","unstructured":"Beers DR, Henkel JS, Zhao W, Wang J, Appel SH (2008) CD4+ T cells support glial neuroprotection, slow disease progression, and modify glial morphology in an animal model of inherited ALS. Proc Natl Acad Sci USA 105:15558\u201315563","journal-title":"Proc Natl Acad Sci USA"},{"key":"741_CR14","doi-asserted-by":"publisher","first-page":"3185","DOI":"10.2174\/092986707782793862","volume":"14","author":"E Beghi","year":"2007","unstructured":"Beghi E, Mennini T, Bendotti C, Bigini P, Logroscino G et al (2007) The heterogeneity of amyotrophic lateral sclerosis: a possible explanation of treatment failure. Curr Med Chem 14:3185\u20133200","journal-title":"Curr Med Chem"},{"key":"741_CR15","doi-asserted-by":"publisher","first-page":"1","DOI":"10.3109\/17482968.2010.502940","volume":"12","author":"E Beghi","year":"2011","unstructured":"Beghi E, Chio A, Couratier P, Esteban J, Hardiman O et al (2011) The epidemiology and treatment of ALS: focus on the heterogeneity of the disease and critical appraisal of therapeutic trials. Amyotroph Lateral Scler 12:1\u201310","journal-title":"Amyotroph Lateral Scler"},{"key":"741_CR16","doi-asserted-by":"publisher","first-page":"1085","DOI":"10.1016\/0028-3908(85)90196-0","volume":"24","author":"J Benavides","year":"1985","unstructured":"Benavides J, Camelin JC, Mitrani N, Flamand F, Uzan A et al (1985) 2-Amino-6-trifluoromethoxy benzothiazole, a possible antagonist of excitatory amino acid neurotransmission-II. Biochemical properties. Neuropharmacology 24:1085\u20131092","journal-title":"Neuropharmacology"},{"key":"741_CR17","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1095\/biolreprod.104.030866","volume":"72","author":"G Berruti","year":"2005","unstructured":"Berruti G, Martegani E (2005) The deubiquitinating enzyme mUBPy interacts with the sperm-specific molecular chaperone MSJ-1: the relation with the proteasome, acrosome, and centrosome in mouse male germ cells. Biol Reprod 72:14\u201321","journal-title":"Biol Reprod"},{"issue":"5","key":"741_CR18","doi-asserted-by":"publisher","first-page":"930","DOI":"10.1095\/biolreprod.109.081679","volume":"82","author":"G Berruti","year":"2010","unstructured":"Berruti G, Ripolone M, Ceriani M (2010) USP8, a regulator of endosomal sorting, is involved in mouse acrosome biogenesis through interaction with the spermatid ESCRT-0 complex and microtubules. Biol Reprod 82:930\u2013939","journal-title":"Biol Reprod"},{"key":"741_CR19","doi-asserted-by":"publisher","first-page":"2291","DOI":"10.1046\/j.1460-9568.2002.02299.x","volume":"16","author":"M Bertamini","year":"2002","unstructured":"Bertamini M, Marzani B, Guarneri R, Guarneri P, Bigini P et al (2002) Mitochondrial oxidative metabolism in motor neuron degeneration (mnd) mouse central nervous system. Eur J Neurosci 16:2291\u20132296","journal-title":"Eur J Neurosci"},{"key":"741_CR20","doi-asserted-by":"publisher","first-page":"1815","DOI":"10.1097\/00001756-200107030-00011","volume":"12","author":"P Bigini","year":"2001","unstructured":"Bigini P, Bastone A, Mennini T (2001) Glutamate transporters in the spinal cord of the wobbler mouse. NeuroReport 12:1815\u20131820","journal-title":"NeuroReport"},{"key":"741_CR21","doi-asserted-by":"publisher","first-page":"334","DOI":"10.1016\/S0304-3940(02)00667-5","volume":"329","author":"P Bigini","year":"2002","unstructured":"Bigini P, Larini S, Pasquali C, Muzio V, Mennini T (2002) Acetyl-l-carnitine shows neuroprotective and neurotrophic activity in primary culture of rat embryo motoneurons. Neurosci Lett 329:334\u2013338","journal-title":"Neurosci Lett"},{"key":"741_CR22","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1186\/1471-2202-7-71","volume":"7","author":"P Bigini","year":"2006","unstructured":"Bigini P, Gardoni F, Barbera S, Cagnotto A, Fumagalli E et al (2006) Expression of AMPA and NMDA receptor subunits in the cervical spinal cord of wobbler mice. BMC Neurosci 7:71","journal-title":"BMC Neurosci"},{"key":"741_CR23","doi-asserted-by":"publisher","first-page":"465","DOI":"10.1016\/j.nbd.2007.11.005","volume":"29","author":"P Bigini","year":"2008","unstructured":"Bigini P, Repici M, Cantarella G, Fumagalli E, Barbera S et al (2008) Recombinant human TNF-binding protein-1 (rhTBP-1) treatment delays both symptoms progression and motor neuron loss in the wobbler mouse. Neurobiol Dis 29:465\u2013476","journal-title":"Neurobiol Dis"},{"key":"741_CR24","doi-asserted-by":"publisher","first-page":"623","DOI":"10.1089\/rej.2011.1197","volume":"14","author":"P Bigini","year":"2011","unstructured":"Bigini P, Veglianese P, Andriolo G, Cova L, Grignaschi G et al (2011) Intracerebroventricular administration of human umbilical cord blood cells delays disease progression in two murine models of motor neuron degeneration. Rejuvenation Res 14:623\u2013639","journal-title":"Rejuvenation Res"},{"key":"741_CR25","doi-asserted-by":"publisher","first-page":"e32326","DOI":"10.1371\/journal.pone.0032326","volume":"7","author":"P Bigini","year":"2012","unstructured":"Bigini P, Diana V, Barbera S, Fumagalli E, Micotti E et al (2012) Longitudinal tracking of human fetal cells labeled with super paramagnetic iron oxide nanoparticles in the brain of mice with motor neuron disease. PLoS One 7:e32326","journal-title":"PLoS One"},{"key":"741_CR26","doi-asserted-by":"publisher","first-page":"20523","DOI":"10.1073\/pnas.1006869107","volume":"107","author":"LG Bilsland","year":"2010","unstructured":"Bilsland LG, Sahai E, Kelly G, Golding M, Greensmith L, Schiavo G (2010) Deficits in axonal transport precede ALS symptoms in vivo. Proc Natl Acad Sci USA 107:20523\u201320528","journal-title":"Proc Natl Acad Sci USA"},{"key":"741_CR27","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1007\/BF00690953","volume":"19","author":"MT Bird","year":"1971","unstructured":"Bird MT, Shuttleworth E Jr, Koestner A, Reinglass J (1971) The wobbler mouse mutant: an animal model of hereditary motor system disease. Acta Neuropathol 19:39\u201350","journal-title":"Acta Neuropathol"},{"key":"741_CR28","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1113\/expphysiol.1982.sp002675","volume":"67","author":"TJ Biscoe","year":"1982","unstructured":"Biscoe TJ, Lewkowicz SJ (1982) Quantitative light and electron microscopic studies on the ventral roots of the wobbler mutant mouse. Q J Exp Physiol 67:543\u2013555","journal-title":"Q J Exp Physiol"},{"key":"741_CR29","doi-asserted-by":"publisher","first-page":"1251","DOI":"10.1002\/glia.20848","volume":"57","author":"D Blackburn","year":"2009","unstructured":"Blackburn D, Sargsyan S, Monk PN, Shaw PJ (2009) Astrocyte function and role in motor neuron disease: a future therapeutic target? Glia 57:1251\u20131264","journal-title":"Glia"},{"key":"741_CR30","doi-asserted-by":"publisher","first-page":"303","DOI":"10.1016\/0006-8993(92)90958-C","volume":"580","author":"B Blondet","year":"1992","unstructured":"Blondet B, Barlovatz-Meimon G, Festoff BW, Soria C, Soria J et al (1992) Plasminogen activators in the neuromuscular system of the wobbler mutant mouse. Brain Res 580:303\u2013310","journal-title":"Brain Res"},{"key":"741_CR31","doi-asserted-by":"publisher","first-page":"53","DOI":"10.1016\/S0006-8993(01)03405-9","volume":"930","author":"B Blondet","year":"2002","unstructured":"Blondet B, Carpentier G, Ait-Ikhlef A, Murawsky M, Rieger F (2002) Motoneuron morphological alterations before and after the onset of the disease in the wobbler mouse. Brain Res 930:53\u201357","journal-title":"Brain Res"},{"key":"741_CR32","doi-asserted-by":"publisher","first-page":"277","DOI":"10.1002\/1098-1136(20010315)33:4<277::AID-GLIA1026>3.0.CO;2-Y","volume":"33","author":"S Boillee","year":"2001","unstructured":"Boillee S, Viala L, Peschanski M, Dreyfus PA (2001) Differential microglial response to progressive neurodegeneration in the murine mutant Wobbler. Glia 33:277\u2013287","journal-title":"Glia"},{"key":"741_CR33","doi-asserted-by":"publisher","first-page":"65","DOI":"10.1385\/MN:28:1:65","volume":"28","author":"S Boillee","year":"2003","unstructured":"Boillee S, Peschanski M, Junier MP (2003) The wobbler mouse: a neurodegeneration jigsaw puzzle. Mol Neurobiol 28:65\u2013106","journal-title":"Mol Neurobiol"},{"key":"741_CR34","doi-asserted-by":"publisher","first-page":"563","DOI":"10.1083\/jcb.200208001","volume":"159","author":"H Bommel","year":"2002","unstructured":"Bommel H, Xie G, Rossoll W, Wiese S, Jablonka S et al (2002) Missense mutation in the tubulin-specific chaperone E (Tbce) gene in the mouse mutant progressive motor neuronopathy, a model of human motoneuron disease. J Cell Biol 159:563\u2013569","journal-title":"J Cell Biol"},{"key":"741_CR35","doi-asserted-by":"publisher","first-page":"302","DOI":"10.1016\/j.neuint.2009.03.013","volume":"55","author":"G Bonanno","year":"2009","unstructured":"Bonanno G, Fumagalli E, Milanese M, Zappettini S, Mennini T (2009) Release of [3H]d-aspartate induced by K\u00a0+\u00a0-stimulation is increased in the cervical spinal cord of the wobbler mouse: a model of motor neuron disease. Neurochem Int 55:302\u2013306","journal-title":"Neurochem Int"},{"key":"741_CR36","doi-asserted-by":"publisher","first-page":"159","DOI":"10.1016\/j.tcb.2010.11.003","volume":"21","author":"JS Bonifacino","year":"2011","unstructured":"Bonifacino JS, Hierro A (2011) Transport according to GARP: receiving retrograde cargo at the trans-Golgi network. Trends Cell Biol 21:159\u2013167","journal-title":"Trends Cell Biol"},{"key":"741_CR37","doi-asserted-by":"publisher","first-page":"583","DOI":"10.1212\/WNL.51.2.583","volume":"51","author":"GD Borasio","year":"1998","unstructured":"Borasio GD, Robberecht W, Leigh PN, Emile J, Guiloff RJ et al (1998) A placebo-controlled trial of insulin-like growth factor-I in amyotrophic lateral sclerosis. European ALS\/IGF-I study group. Neurology 51:583\u2013586","journal-title":"Neurology"},{"key":"741_CR38","doi-asserted-by":"publisher","first-page":"429","DOI":"10.1016\/S0361-9230(99)00024-6","volume":"48","author":"P Bose","year":"1999","unstructured":"Bose P, Fielding R, Vacca-Galloway LL (1999) Effects of assisted feeding on wobbler mouse motoneuron disease and on serotonergic and peptidergic sprouting in the cervical spinal ventral horn. Brain Res Bull 48:429\u2013439","journal-title":"Brain Res Bull"},{"key":"741_CR39","doi-asserted-by":"publisher","first-page":"723","DOI":"10.1146\/annurev.neuro.27.070203.144244","volume":"27","author":"LI Bruijn","year":"2004","unstructured":"Bruijn LI, Miller TM, Cleveland DW (2004) Unraveling the mechanisms involved in motor neuron degeneration in ALS. Annu Rev Neurosci 27:723\u2013749","journal-title":"Annu Rev Neurosci"},{"key":"741_CR40","doi-asserted-by":"publisher","first-page":"56","DOI":"10.1016\/j.brainres.2007.12.014","volume":"1195","author":"F Bruzzone","year":"2008","unstructured":"Bruzzone F, Vallarino M, Berruti G, Angelini C (2008) Expression of the deubiquitinating enzyme mUBPy in the mouse brain. Brain Res 1195:56\u201366","journal-title":"Brain Res"},{"key":"741_CR41","doi-asserted-by":"publisher","first-page":"401","DOI":"10.2119\/molmed.2011.00123","volume":"18","author":"L Canzi","year":"2012","unstructured":"Canzi L, Castellaneta V, Navone S, Nava S, Dossena M et al (2012) Human skeletal muscle stem cell antiinflammatory activity ameliorates clinical outcome in amyotrophic lateral sclerosis models. Mol Med 18:401\u2013411","journal-title":"Mol Med"},{"key":"741_CR42","doi-asserted-by":"publisher","first-page":"e2849","DOI":"10.1371\/journal.pone.0002849","volume":"3","author":"Y Chang","year":"2008","unstructured":"Chang Y, Kong Q, Shan X, Tian G, Ilieva H et al (2008) Messenger RNA oxidation occurs early in disease pathogenesis and promotes motor neuron degeneration in ALS. PLoS ONE 3:e2849","journal-title":"PLoS ONE"},{"key":"741_CR43","doi-asserted-by":"publisher","first-page":"1128","DOI":"10.1086\/421054","volume":"74","author":"YZ Chen","year":"2004","unstructured":"Chen YZ, Bennett CL, Huynh HM, Blair IP, Puls I et al (2004) DNA\/RNA helicase gene mutations in a form of juvenile amyotrophic lateral sclerosis (ALS4). Am J Hum Genet 74:1128\u20131135","journal-title":"Am J Hum Genet"},{"key":"741_CR44","doi-asserted-by":"publisher","first-page":"40266","DOI":"10.1074\/jbc.M110.161398","volume":"285","author":"HJ Chen","year":"2010","unstructured":"Chen HJ, Anagnostou G, Chai A, Withers J, Morris A et al (2010) Characterization of the properties of a novel mutation in VAPB in familial amyotrophic lateral sclerosis. J Biol Chem 285:40266\u201340281","journal-title":"J Biol Chem"},{"key":"741_CR45","doi-asserted-by":"publisher","first-page":"85","DOI":"10.1016\/j.ajhg.2008.12.010","volume":"84","author":"CY Chow","year":"2009","unstructured":"Chow CY, Landers JE, Bergren SK, Sapp PC, Grant AE et al (2009) Deleterious variants of FIG4, a phosphoinositide phosphatase, in patients with ALS. Am J Hum Genet 84:85\u201388","journal-title":"Am J Hum Genet"},{"key":"741_CR46","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 et al (2003) Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice. Science 302:113\u2013117","journal-title":"Science"},{"key":"741_CR47","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1091\/mbc.11.1.305","volume":"11","author":"E Conibear","year":"2000","unstructured":"Conibear E, Stevens TH (2000) Vps52p, Vps53p, and Vps54p form a novel multisubunit complex required for protein sorting at the yeast late Golgi. Mol Biol Cell 11:305\u2013323","journal-title":"Mol Biol Cell"},{"key":"741_CR48","doi-asserted-by":"publisher","first-page":"1610","DOI":"10.1091\/mbc.E02-10-0654","volume":"14","author":"E Conibear","year":"2003","unstructured":"Conibear E, Cleck JN, Stevens TH (2003) Vps51p mediates the association of the GARP (Vps52\/53\/54) complex with the late Golgi t-SNARE Tlg1p. Mol Biol Cell 14:1610\u20131623","journal-title":"Mol Biol Cell"},{"key":"741_CR49","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1016\/0092-8674(93)90158-M","volume":"73","author":"F Cote","year":"1993","unstructured":"Cote F, Collard JF, Julien JP (1993) Progressive neuronopathy in transgenic mice expressing the human neurofilament heavy gene: a mouse model of amyotrophic lateral sclerosis. Cell 73:35\u201346","journal-title":"Cell"},{"key":"741_CR50","doi-asserted-by":"publisher","first-page":"e9872","DOI":"10.1371\/journal.pone.0009872","volume":"5","author":"LE Cox","year":"2010","unstructured":"Cox LE, Ferraiuolo L, Goodall EF, Heath PR, Higginbottom A et al (2010) Mutations in CHMP2B in lower motor neuron predominant amyotrophic lateral sclerosis (ALS). PLoS One 5:e9872","journal-title":"PLoS One"},{"key":"741_CR51","doi-asserted-by":"publisher","first-page":"1349","DOI":"10.1111\/j.1471-4159.2006.03619.x","volume":"96","author":"M Damiano","year":"2006","unstructured":"Damiano M, Starkov AA, Petri S, Kipiani K, Kiaei M et al (2006) Neural mitochondrial Ca2+ capacity impairment precedes the onset of motor symptoms in G93A Cu\/Zn-superoxide dismutase mutant mice. J Neurochem 96:1349\u20131361","journal-title":"J Neurochem"},{"key":"741_CR52","doi-asserted-by":"publisher","first-page":"412","DOI":"10.1016\/S0014-4886(03)00091-8","volume":"182","author":"KR Dave","year":"2003","unstructured":"Dave KR, Bradley WG, Perez-Pinzon MA (2003a) Early mitochondrial dysfunction occurs in motor cortex and spinal cord at the onset of disease in the Wobbler mouse. Exp Neurol 182:412\u2013420","journal-title":"Exp Neurol"},{"key":"741_CR53","doi-asserted-by":"publisher","first-page":"113","DOI":"10.1016\/S0306-4522(03)00244-6","volume":"120","author":"KR Dave","year":"2003","unstructured":"Dave KR, Prado R, Busto R, Raval AP, Bradley WG et al (2003b) Hyperbaric oxygen therapy protects against mitochondrial dysfunction and delays onset of motor neuron disease in Wobbler mice. Neuroscience 120:113\u2013120","journal-title":"Neuroscience"},{"key":"741_CR54","doi-asserted-by":"publisher","first-page":"126","DOI":"10.1016\/j.nbd.2004.08.017","volume":"18","author":"KR Dave","year":"2005","unstructured":"Dave KR, Raval AP, Purroy J, Kirkinezos IG, Moraes CT et al (2005) Aberrant deltaPKC activation in the spinal cord of Wobbler mouse: a model of motor neuron disease. Neurobiol Dis 18:126\u2013133","journal-title":"Neurobiol Dis"},{"key":"741_CR55","doi-asserted-by":"crossref","first-page":"971","DOI":"10.2119\/molmed.2012.00020","volume":"18","author":"M Paola De","year":"2012","unstructured":"De Paola M, Mariani A, Bigini P, Peviani M, Ferrara G et al (2012) Neuroprotective effects of toll-like receptor 4 antagonism in spinal cord cultures and in a mouse model of motor neuron degeneration. Mol Med 18:971\u2013981","journal-title":"Mol Med"},{"key":"741_CR56","doi-asserted-by":"publisher","first-page":"2720","DOI":"10.1093\/hmg\/ddm226","volume":"16","author":"KJ Vos De","year":"2007","unstructured":"De Vos KJ, Chapman AL, Tennant ME, Manser C, Tudor EL et al (2007) Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content. Hum Mol Genet 16:2720\u20132728","journal-title":"Hum Mol Genet"},{"key":"741_CR57","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, Mackenzie IR, Boeve BF, Boxer AL, Baker 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":"741_CR58","doi-asserted-by":"publisher","first-page":"211","DOI":"10.1038\/nature10353","volume":"477","author":"HX Deng","year":"2011","unstructured":"Deng HX, Chen W, Hong ST, Boycott KM, Gorrie GH 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":"741_CR59","doi-asserted-by":"publisher","first-page":"185","DOI":"10.1111\/j.1471-4159.2012.07753.x","volume":"122","author":"MC Deniselle","year":"2012","unstructured":"Deniselle MC, Carreras MC, Garay L, Gargiulo-Monachelli G, Meyer M et al (2012) Progesterone prevents mitochondrial dysfunction in the spinal cord of wobbler mice. J Neurochem 122:185\u2013195","journal-title":"J Neurochem"},{"key":"741_CR60","doi-asserted-by":"publisher","first-page":"745","DOI":"10.1016\/j.neuroscience.2008.10.030","volume":"158","author":"JS Dennis","year":"2009","unstructured":"Dennis JS, Citron BA (2009) Wobbler mice modeling motor neuron disease display elevated transactive response DNA binding protein. Neuroscience 158:745\u2013750","journal-title":"Neuroscience"},{"key":"741_CR61","doi-asserted-by":"publisher","first-page":"9595","DOI":"10.1073\/pnas.0510197103","volume":"103","author":"RS Devon","year":"2006","unstructured":"Devon RS, Orban PC, Gerrow K, Barbieri MA, Schwab C et al (2006) Als2-deficient mice exhibit disturbances in endosome trafficking associated with motor behavioral abnormalities. Proc Natl Acad Sci USA 103:9595\u20139600","journal-title":"Proc Natl Acad Sci USA"},{"key":"741_CR62","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":"741_CR63","doi-asserted-by":"publisher","first-page":"163","DOI":"10.1016\/j.expneurol.2010.06.008","volume":"225","author":"V Diana","year":"2010","unstructured":"Diana V, Ottolina A, Botti F, Fumagalli E, Calcagno E et al (2010) Neural precursor-derived astrocytes of wobbler mice induce apoptotic death of motor neurons through reduced glutamate uptake. Exp Neurol 225:163\u2013172","journal-title":"Exp Neurol"},{"key":"741_CR64","doi-asserted-by":"publisher","first-page":"2841","DOI":"10.1038\/emboj.2010.143","volume":"29","author":"D Dormann","year":"2010","unstructured":"Dormann D, Rodde R, Edbauer D, Bentmann E, Fischer I et al (2010) ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import. EMBO J 29:2841\u20132857","journal-title":"EMBO J"},{"key":"741_CR65","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":"741_CR66","doi-asserted-by":"publisher","first-page":"535","DOI":"10.1136\/jnnp.31.6.535","volume":"31","author":"LW Duchen","year":"1968","unstructured":"Duchen LW, Strich SJ (1968) An hereditary motor neuron disease with progressive denervation of muscle in mouse: the mutant \u201cwobbler\u201d. J Neurol Neurosurg Psychiat 31:535\u2013542","journal-title":"J Neurol Neurosurg Psychiat"},{"key":"741_CR67","doi-asserted-by":"publisher","first-page":"336","DOI":"10.1111\/j.1365-2990.2011.01166.x","volume":"37","author":"LM Duffy","year":"2011","unstructured":"Duffy LM, Chapman AL, Shaw PJ, Grierson AJ (2011) Review: the role of mitochondria in the pathogenesis of amyotrophic lateral sclerosis. Neuropathol Appl Neurobiol 37:336\u2013352","journal-title":"Neuropathol Appl Neurobiol"},{"key":"741_CR68","first-page":"22","volume":"15","author":"DS Falconer","year":"1956","unstructured":"Falconer DS (1956) Wobbler (wr). Mouse News Lett 15:22","journal-title":"Mouse News Lett"},{"key":"741_CR69","doi-asserted-by":"publisher","first-page":"616","DOI":"10.1038\/nrneurol.2011.152","volume":"7","author":"L Ferraiuolo","year":"2011","unstructured":"Ferraiuolo L, Kirby J, Grierson AJ, Sendtner M, Shaw PJ (2011) Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis. Nat Rev Neurol 7:616\u2013630","journal-title":"Nat Rev Neurol"},{"key":"741_CR70","first-page":"797","volume":"19","author":"PS Fitzmaurice","year":"1996","unstructured":"Fitzmaurice PS, Shaw IC, Kleiner HE, Miller RT, Monks TJ et al (1996) Evidence for DNA damage in amyotrophic lateral sclerosis. Muscle Nerve 19:797\u2013798","journal-title":"Muscle Nerve"},{"key":"741_CR71","doi-asserted-by":"publisher","first-page":"1596","DOI":"10.1212\/WNL.0b013e3182563b57","volume":"78","author":"BR Foerster","year":"2012","unstructured":"Foerster BR, Callaghan BC, Petrou M, Edden RA, Chenevert TL, Feldman EL (2012) Decreased motor cortex gamma-aminobutyric acid in amyotrophic lateral sclerosis. Neurology 78:1596\u20131600","journal-title":"Neurology"},{"key":"741_CR72","doi-asserted-by":"publisher","first-page":"2481","DOI":"10.1046\/j.1460-9568.1998.00273.x","volume":"10","author":"AE Fray","year":"1998","unstructured":"Fray AE, Ince PG, Banner SJ, Milton ID, Usher PA et al (1998) The expression of the glial glutamate transporter protein EAAT2 in motor neuron disease: an immunohistochemical study. Eur J Neurosci 10:2481\u20132489","journal-title":"Eur J Neurosci"},{"key":"741_CR73","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1186\/1471-2156-3-14","volume":"3","author":"S Fuchs","year":"2002","unstructured":"Fuchs S, Resch K, Thiel C, Ulbrich M, Platzer M et al (2002) Comparative transcription map of the wobbler critical region on mouse chromosome 11 and the homologous region on human chromosome 2p13-14. BMC Genet 3:14","journal-title":"BMC Genet"},{"key":"741_CR74","doi-asserted-by":"publisher","first-page":"114","DOI":"10.1016\/j.expneurol.2005.11.010","volume":"198","author":"E Fumagalli","year":"2006","unstructured":"Fumagalli E, Bigini P, Barbera S, De Paola M, Mennini T (2006) Riluzole, unlike the AMPA antagonist RPR119990, reduces motor impairment and partially prevents motoneuron death in the wobbler mouse, a model of neurodegenerative disease. Exp Neurol 198:114\u2013128","journal-title":"Exp Neurol"},{"key":"741_CR75","doi-asserted-by":"publisher","first-page":"e2494","DOI":"10.1371\/journal.pone.0002494","volume":"3","author":"S Garbuzova-Davis","year":"2008","unstructured":"Garbuzova-Davis S, Sanberg CD, Kuzmin-Nichols N, Willing AE, Gemma C et al (2008) Human umbilical cord blood treatment in a mouse model of ALS: optimization of cell dose. PLoS One 3:e2494","journal-title":"PLoS One"},{"issue":"Suppl 1","key":"741_CR76","doi-asserted-by":"publisher","first-page":"S37","DOI":"10.1038\/sj.cdd.4401450","volume":"11","author":"P Ghezzi","year":"2004","unstructured":"Ghezzi P, Brines M (2004) Erythropoietin as an antiapoptotic, tissue-protective cytokine. Cell Death Differ 11(Suppl 1):S37\u2013S44","journal-title":"Cell Death Differ"},{"key":"741_CR77","doi-asserted-by":"publisher","first-page":"755","DOI":"10.1083\/jcb.200308038","volume":"163","author":"P Ghosh","year":"2003","unstructured":"Ghosh P, Griffith J, Geuze HJ, Kornfeld S (2003) Mammalian GGAs act together to sort mannose 6-phosphate receptors. J Cell Biol 163:755\u2013766","journal-title":"J Cell Biol"},{"key":"741_CR78","doi-asserted-by":"publisher","first-page":"351","DOI":"10.1111\/j.1750-3639.2009.00284.x","volume":"20","author":"MT Giordana","year":"2010","unstructured":"Giordana MT, Piccinini M, Grifoni S, De Marco G, Vercellino M et al (2010) TDP-43 redistribution is an early event in sporadic amyotrophic lateral sclerosis. Brain Pathol 20:351\u2013360","journal-title":"Brain Pathol"},{"key":"741_CR79","doi-asserted-by":"publisher","first-page":"61","DOI":"10.1007\/BF02578387","volume":"16","author":"MC Gonzalez Deniselle","year":"1996","unstructured":"Gonzalez Deniselle MC, Gonzalez SL, Piroli GG, Lima AE, De Nicola AF (1996) The 21-aminosteroid U-74389F increases the number of glial fibrillary acidic protein-expressing astrocytes in the spinal cord of control and Wobbler mice. Cell Mol Neurobiol 16:61\u201372","journal-title":"Cell Mol Neurobiol"},{"key":"741_CR80","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1023\/A:1020918310281","volume":"24","author":"MC Gonzalez Deniselle","year":"1999","unstructured":"Gonzalez Deniselle MC, Gonzalez SL, Lima AE, Wilkin G, De Nicola AF (1999) The 21-aminosteroid U-74389F attenuates hyperexpression of GAP-43 and NADPH-diaphorase in the spinal cord of wobbler mouse, a model for amyotrophic lateral sclerosis. Neurochem Res 24:1\u20138","journal-title":"Neurochem Res"},{"key":"741_CR81","doi-asserted-by":"publisher","first-page":"457","DOI":"10.1006\/nbdi.2002.0564","volume":"11","author":"MC Gonzalez Deniselle","year":"2002","unstructured":"Gonzalez Deniselle MC, Lopez-Costa JJ, Saavedra JP, Pietranera L, Gonzalez SL et al (2002) Progesterone neuroprotection in the wobbler mouse, a genetic model of spinal cord motor neuron disease. Neurobiol Dis 11:457\u2013468","journal-title":"Neurobiol Dis"},{"key":"741_CR82","doi-asserted-by":"publisher","first-page":"518","DOI":"10.1016\/j.expneurol.2005.06.015","volume":"195","author":"MC Gonzalez Deniselle","year":"2005","unstructured":"Gonzalez Deniselle MC, Garay L, Gonzalez S, Guennoun R, Schumacher M, De Nicola AF (2005) Progesterone restores retrograde labeling of cervical motoneurons in Wobbler mouse motoneuron disease. Exp Neurol 195:518\u2013523","journal-title":"Exp Neurol"},{"key":"741_CR83","doi-asserted-by":"publisher","first-page":"406","DOI":"10.1016\/j.expneurol.2006.08.019","volume":"203","author":"MC Gonzalez Deniselle","year":"2007","unstructured":"Gonzalez Deniselle MC, Garay L, Gonzalez S, Saravia F, Labombarda F et al (2007) Progesterone modulates brain-derived neurotrophic factor and choline acetyltransferase in degenerating Wobbler motoneurons. Exp Neurol 203:406\u2013414","journal-title":"Exp Neurol"},{"key":"741_CR84","doi-asserted-by":"publisher","first-page":"559","DOI":"10.1002\/(SICI)1097-4547(19980301)51:5<559::AID-JNR2>3.0.CO;2-E","volume":"51","author":"A Gorio","year":"1998","unstructured":"Gorio A, Vergani L, Lesma E, Di Giulio AM (1998) Neuroprotection, neuroregeneration, and interaction with insulin-like growth factor-I: novel non-anticoagulant action of glycosaminoglycans. J Neurosci Res 51:559\u2013562","journal-title":"J Neurosci Res"},{"key":"741_CR85","doi-asserted-by":"publisher","first-page":"194","DOI":"10.1046\/j.1471-4159.2002.00830.x","volume":"81","author":"A Gorio","year":"2002","unstructured":"Gorio A, Lesma E, Madaschi L, Di Giulio AM (2002) Co-administration of IGF-I and glycosaminoglycans greatly delays motor neurone disease and affects IGF-I expression in the wobbler mouse: a long-term study. J Neurochem 81:194\u2013202","journal-title":"J Neurochem"},{"key":"741_CR86","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 et al (2006) ANG mutations segregate with familial and \u2018sporadic\u2019 amyotrophic lateral sclerosis. Nat Genet 38:411\u2013413","journal-title":"Nat Genet"},{"key":"741_CR87","doi-asserted-by":"publisher","first-page":"224","DOI":"10.1001\/archneurol.2009.329","volume":"67","author":"EJ Groen","year":"2010","unstructured":"Groen EJ, van Es MA, van Vught PW, Spliet WG, van Engelen-Lee J et al (2010) FUS mutations in familial amyotrophic lateral sclerosis in the Netherlands. Arch Neurol 67:224\u2013230","journal-title":"Arch Neurol"},{"key":"741_CR88","doi-asserted-by":"publisher","first-page":"2691","DOI":"10.1093\/hmg\/ddn171","volume":"17","author":"F Gros-Louis","year":"2008","unstructured":"Gros-Louis F, Kriz J, Kabashi E, McDearmid J, Millecamps S et al (2008) Als2 mRNA splicing variants detected in KO mice rescue severe motor dysfunction phenotype in Als2 knock-down zebrafish. Hum Mol Genet 17:2691\u20132702","journal-title":"Hum Mol Genet"},{"key":"741_CR89","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 et al (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":"741_CR90","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 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":"741_CR91","doi-asserted-by":"publisher","first-page":"251","DOI":"10.1086\/337945","volume":"70","author":"CK Hand","year":"2002","unstructured":"Hand CK, Khoris J, Salachas F, Gros-Louis F, Lopes AA et al (2002) A novel locus for familial amyotrophic lateral sclerosis, on chromosome 18q. Am J Hum Genet 70:251\u2013256","journal-title":"Am J Hum Genet"},{"key":"741_CR92","doi-asserted-by":"publisher","first-page":"77","DOI":"10.1111\/j.1432-0436.1991.tb00225.x","volume":"47","author":"P Heimann","year":"1991","unstructured":"Heimann P, Laage S, Jockusch H (1991) Defect of sperm assembly in a neurological mutant of the mouse, wobbler (WR). Differentiation 47:77\u201383","journal-title":"Differentiation"},{"key":"741_CR93","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1007\/s100480050052","volume":"2","author":"A Hentati","year":"1998","unstructured":"Hentati A, Ouahchi K, Pericak-Vance MA, Nijhawan D, Ahmad A et al (1998) Linkage of a commoner form of recessive amyotrophic lateral sclerosis to chromosome 15q15\u2013q22 markers. Neurogenetics 2:55\u201360","journal-title":"Neurogenetics"},{"key":"741_CR94","doi-asserted-by":"publisher","first-page":"455","DOI":"10.1001\/archneurol.2010.52","volume":"67","author":"C Hewitt","year":"2010","unstructured":"Hewitt C, Kirby J, Highley JR, Hartley JA, Hibberd R et al (2010) Novel FUS\/TLS mutations and pathology in familial and sporadic amyotrophic lateral sclerosis. Arch Neurol 67:455\u2013461","journal-title":"Arch Neurol"},{"key":"741_CR95","first-page":"742","volume":"21","author":"E Heytens","year":"2010","unstructured":"Heytens E, Schmitt-John T, Moser JM, Mand\u00f8e Jensen N, Soleimani R et al (2010) Reduced fertilization after ICSI and PLCzeta in sperm from the Wobbler mouse. RBM-online 21:742\u2013749","journal-title":"RBM-online"},{"key":"741_CR96","doi-asserted-by":"publisher","first-page":"461","DOI":"10.1097\/00005072-198409000-00001","volume":"43","author":"A Hirano","year":"1984","unstructured":"Hirano A, Donnenfeld H, Sasaki S, Nakano I (1984) Fine structural observations of neurofilamentous changes in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 43:461\u2013470","journal-title":"J Neuropathol Exp Neurol"},{"key":"741_CR97","doi-asserted-by":"publisher","first-page":"347","DOI":"10.1083\/jcb.200310015","volume":"165","author":"J Hitomi","year":"2004","unstructured":"Hitomi J, Katayama T, Eguchi Y, Kudo T, Taniguchi M et al (2004) Involvement of caspase-4 in endoplasmic reticulum stress-induced apoptosis and Abeta-induced cell death. J Cell Biol 165:347\u2013356","journal-title":"J Cell Biol"},{"key":"741_CR98","doi-asserted-by":"publisher","first-page":"1344","DOI":"10.1002\/mus.880181122","volume":"18","author":"K Ikeda","year":"1995","unstructured":"Ikeda K, Iwasaki Y, Tagaya N, Shiojima T, Kinoshita M (1995a) Neuroprotective effect of cholinergic differentiation factor\/leukemia inhibitory factor on wobbler murine motor neuron disease. Muscle Nerve 18:1344\u20131347","journal-title":"Muscle Nerve"},{"key":"741_CR99","doi-asserted-by":"publisher","first-page":"383","DOI":"10.1016\/0960-8966(95)00003-6","volume":"5","author":"K Ikeda","year":"1995","unstructured":"Ikeda K, Kinoshita M, Iwasaki Y, Tagaya N, Shiojima T (1995b) Lecithinized superoxide dismutase retards wobbler mouse motoneuron disease. Neuromuscul Disord 5:383\u2013390","journal-title":"Neuromuscul Disord"},{"key":"741_CR100","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1016\/0006-8993(96)00316-2","volume":"726","author":"K Ikeda","year":"1996","unstructured":"Ikeda K, Kinoshita M, Tagaya N, Shiojima T, Taga T et al (1996) Coadministration of interleukin-6 (IL-6) and soluble IL-6 receptor delays progression of wobbler mouse motor neuron disease. Brain Res 726:91\u201397","journal-title":"Brain Res"},{"key":"741_CR101","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1016\/S0022-510X(98)00224-X","volume":"160","author":"K Ikeda","year":"1998","unstructured":"Ikeda K, Iwasaki Y, Kinoshita M (1998) Neuronal nitric oxide synthase inhibitor, 7-nitroindazole, delays motor dysfunction and spinal motoneuron degeneration in the wobbler mouse. J Neurol Sci 160:9\u201315","journal-title":"J Neurol Sci"},{"key":"741_CR102","doi-asserted-by":"publisher","first-page":"207","DOI":"10.1080\/14660820050515205","volume":"1","author":"K Ikeda","year":"2000","unstructured":"Ikeda K, Iwasaki Y, Kinoshita M (2000a) Oral administration of creatine monohydrate retards progression of motor neuron disease in the wobbler mouse. Amyotroph Lateral Scler Other Motor Neuron Disord 1:207\u2013212","journal-title":"Amyotroph Lateral Scler Other Motor Neuron Disord"},{"key":"741_CR103","doi-asserted-by":"publisher","first-page":"84","DOI":"10.1016\/S0006-8993(99)02427-0","volume":"858","author":"K Ikeda","year":"2000","unstructured":"Ikeda K, Iwasaki Y, Kinoshita M, Marubuchi S, Ono S (2000b) T-588, a novel neuroprotective agent, delays progression of neuromuscular dysfunction in wobbler mouse motoneuron disease. Brain Res 858:84\u201391","journal-title":"Brain Res"},{"key":"741_CR104","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1111\/j.1365-2990.1993.tb00443.x","volume":"19","author":"P Ince","year":"1993","unstructured":"Ince P, Stout N, Shaw P, Slade J, Hunziker W et al (1993) Parvalbumin and Calbindin D-28k in the human motor system and in motor neuron disease. Neuropathol Appl Neurobiol 19:291\u2013299","journal-title":"Neuropathol Appl Neurobiol"},{"key":"741_CR105","doi-asserted-by":"publisher","first-page":"895","DOI":"10.1097\/00005072-199810000-00002","volume":"57","author":"PG Ince","year":"1998","unstructured":"Ince PG, Tomkins J, Slade JY, Thatcher NM, Shaw PJ (1998) Amyotrophic lateral sclerosis associated with genetic abnormalities in the gene encoding Cu\/Zn superoxide dismutase: molecular pathology of five new cases, and comparison with previous reports and 73 sporadic cases of ALS. J Neuropathol Exp Neurol 57:895\u2013904","journal-title":"J Neuropathol Exp Neurol"},{"key":"741_CR106","doi-asserted-by":"publisher","first-page":"195","DOI":"10.1016\/S0006-8993(02)02881-0","volume":"944","author":"T Ishiyama","year":"2002","unstructured":"Ishiyama T, Ogo H, Wong V, Klinkosz B, Noguchi H et al (2002) Methionine-free brain-derived neurotrophic factor in wobbler mouse motor neuron disease: dose-related effects and comparison with the methionyl form. Brain Res 944:195\u2013199","journal-title":"Brain Res"},{"key":"741_CR107","doi-asserted-by":"publisher","first-page":"226","DOI":"10.1016\/j.brainres.2004.06.002","volume":"1019","author":"T Ishiyama","year":"2004","unstructured":"Ishiyama T, Okada R, Nishibe H, Mitsumoto H, Nakayama C (2004) Riluzole slows the progression of neuromuscular dysfunction in the wobbler mouse motor neuron disease. Brain Res 1019:226\u2013236","journal-title":"Brain Res"},{"key":"741_CR108","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1002\/ana.22246","volume":"69","author":"D Ito","year":"2011","unstructured":"Ito D, Seki M, Tsunoda Y, Uchiyama H, Suzuki N (2011) Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS\/TLS. Ann Neurol 69:152\u2013162","journal-title":"Ann Neurol"},{"key":"741_CR109","doi-asserted-by":"publisher","first-page":"405","DOI":"10.3109\/17482960902870993","volume":"10","author":"K Iwamoto","year":"2009","unstructured":"Iwamoto K, Yoshii Y, Ikeda K (2009) Atorvastatin treatment attenuates motor neuron degeneration in wobbler mice. Amyotroph Lateral Scler 10:405\u2013409","journal-title":"Amyotroph Lateral Scler"},{"key":"741_CR110","doi-asserted-by":"publisher","first-page":"225","DOI":"10.1023\/A:1014884504879","volume":"27","author":"Y Iwasaki","year":"2002","unstructured":"Iwasaki Y, Ikeda K, Ichikawa Y, Igarashi O, Kinoshita M et al (2002) T-588 enhances neurite outgrowth and choline acetyltransferase in cultured rat spinal ventral horn neurons. Neurochem Res 27:225\u2013228","journal-title":"Neurochem Res"},{"key":"741_CR111","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, Mandrioli J, Benatar M, Abramzon Y, Van Deerlin VM et al (2010) Exome sequencing reveals VCP mutations as a cause of familial ALS. Neuron 68:857\u2013864","journal-title":"Neuron"},{"key":"741_CR112","doi-asserted-by":"publisher","first-page":"671","DOI":"10.1093\/hmg\/ddp534","volume":"19","author":"E Kabashi","year":"2010","unstructured":"Kabashi E, Lin L, Tradewell ML, Dion PA, Bercier V et al (2010) Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo. Hum Mol Genet 19:671\u2013683","journal-title":"Hum Mol Genet"},{"key":"741_CR113","doi-asserted-by":"publisher","first-page":"81","DOI":"10.1007\/s12035-009-8054-3","volume":"39","author":"K Kanekura","year":"2009","unstructured":"Kanekura K, Suzuki H, Aiso S, Matsuoka M (2009) ER stress and unfolded protein response in amyotrophic lateral sclerosis. Mol Neurobiol 39:81\u201389","journal-title":"Mol Neurobiol"},{"key":"741_CR114","doi-asserted-by":"crossref","first-page":"1389","DOI":"10.1172\/JCI0216886","volume":"110","author":"RJ Kaufman","year":"2002","unstructured":"Kaufman RJ (2002) Orchestrating the unfolded protein response in health and disease. J Clin Invest 110:1389\u20131398","journal-title":"J Clin Invest"},{"key":"741_CR115","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1016\/0888-7543(92)90199-3","volume":"13","author":"K Kaupmann","year":"1992","unstructured":"Kaupmann K, Simon-Chazottes D, Guenet JL, Jockusch H (1992) Wobbler, a mutation affecting motoneuron survival and gonadal functions in the mouse, maps to proximal chromosome 11. Genomics 13:39\u201343","journal-title":"Genomics"},{"key":"741_CR116","doi-asserted-by":"publisher","first-page":"74","DOI":"10.1016\/j.expneurol.2007.01.036","volume":"205","author":"M Kiaei","year":"2007","unstructured":"Kiaei M, Kipiani K, Calingasan NY, Wille E, Chen J et al (2007) Matrix metalloproteinase-9 regulates TNF-alpha and FasL expression in neuronal, glial cells and its absence extends life in a transgenic mouse model of amyotrophic lateral sclerosis. Exp Neurol 205:74\u201381","journal-title":"Exp Neurol"},{"key":"741_CR117","doi-asserted-by":"publisher","first-page":"242","DOI":"10.1006\/geno.1997.4789","volume":"43","author":"D Korthaus","year":"1997","unstructured":"Korthaus D, Wedemeyer N, Lengeling A, Ronsiek M, Jockusch H, Schmitt-John T (1997) Integrated radiation hybrid map of human chromosome 2p13: possible involvement of dynactin in neuromuscular diseases. Genomics 43:242\u2013244","journal-title":"Genomics"},{"key":"741_CR118","doi-asserted-by":"publisher","first-page":"409","DOI":"10.1007\/s00401-010-0659-0","volume":"119","author":"BC Kraemer","year":"2010","unstructured":"Kraemer BC, Schuck T, Wheeler JM, Robinson LC, Trojanowski JQ et al (2010) Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis. Acta Neuropathol 119:409\u2013419","journal-title":"Acta Neuropathol"},{"key":"741_CR119","doi-asserted-by":"publisher","first-page":"142","DOI":"10.1016\/0006-8993(91)90925-L","volume":"551","author":"C Krieger","year":"1991","unstructured":"Krieger C, Perry TL, Hansen S, Mitsumoto H (1991) The wobbler mouse: amino acid contents in brain and spinal cord. Brain Res 551:142\u2013144","journal-title":"Brain Res"},{"key":"741_CR120","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1017\/S0317167100041652","volume":"19","author":"C Krieger","year":"1992","unstructured":"Krieger C, Perry TL, Hansen S, Mitsumoto H, Honore T (1992) Excitatory amino acid receptor antagonist in murine motoneuron disease (the wobbler mouse). Can J Neurol Sci 19:462\u2013465","journal-title":"Can J Neurol Sci"},{"key":"741_CR121","doi-asserted-by":"publisher","first-page":"771","DOI":"10.1111\/j.1468-1331.2009.02560.x","volume":"16","author":"J Kuhle","year":"2009","unstructured":"Kuhle J, Lindberg RL, Regeniter A, Mehling M, Steck AJ et al (2009) Increased levels of inflammatory chemokines in amyotrophic lateral sclerosis. Eur J Neurol 16:771\u2013774","journal-title":"Eur J Neurol"},{"issue":"Suppl 1","key":"741_CR122","doi-asserted-by":"publisher","first-page":"S106","DOI":"10.1016\/S0022-510X(98)00208-1","volume":"160","author":"JB Kurek","year":"1998","unstructured":"Kurek JB, Radford AJ, Crump DE, Bower JJ, Feeney SJ et al (1998) LIF (AM424), a promising growth factor for the treatment of ALS. J Neurol Sci 160(Suppl 1):S106\u2013S113","journal-title":"J Neurol Sci"},{"key":"741_CR123","doi-asserted-by":"publisher","first-page":"182","DOI":"10.1111\/j.1440-1789.2009.01090.x","volume":"30","author":"S Kwak","year":"2010","unstructured":"Kwak S, Hideyama T, Yamashita T, Aizawa H (2010) AMPA receptor-mediated neuronal death in sporadic ALS. Neuropathology 30:182\u2013188","journal-title":"Neuropathology"},{"key":"741_CR124","doi-asserted-by":"publisher","first-page":"1205","DOI":"10.1126\/science.1166066","volume":"323","author":"TJ Kwiatkowski Jr","year":"2009","unstructured":"Kwiatkowski TJ Jr, Bosco DA, Leclerc AL, Tamrazian E, Vanderburg CR 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":"741_CR125","doi-asserted-by":"publisher","first-page":"11798","DOI":"10.1523\/JNEUROSCI.2084-06.2006","volume":"26","author":"C Lai","year":"2006","unstructured":"Lai C, Xie C, McCormack SG, Chiang HC, Michalak MK et al (2006) Amyotrophic lateral sclerosis 2-deficiency leads to neuronal degeneration in amyotrophic lateral sclerosis through altered AMPA receptor trafficking. J Neurosci 26:11798\u201311806","journal-title":"J Neurosci"},{"key":"741_CR126","doi-asserted-by":"publisher","first-page":"127","DOI":"10.1016\/0006-8993(87)91363-1","volume":"404","author":"JH LaVail","year":"1987","unstructured":"LaVail JH, Koo EH, Dekker NP (1987) Motoneuron loss in the abducens nucleus of wobbler mice. Brain Res 404:127\u2013132","journal-title":"Brain Res"},{"key":"741_CR127","doi-asserted-by":"publisher","first-page":"267","DOI":"10.1530\/jrf.0.0580267","volume":"58","author":"JE Leestma","year":"1980","unstructured":"Leestma JE, Sepsenwol S (1980) Sperm tail axoneme alterations in the wobbler mouse. J Reprod Fertil 58:267\u2013270","journal-title":"J Reprod Fertil"},{"key":"741_CR128","doi-asserted-by":"publisher","first-page":"2359","DOI":"10.1093\/hmg\/ddm193","volume":"16","author":"R Lemmens","year":"2007","unstructured":"Lemmens R, Van Hoecke A, Hersmus N, Geelen V, D\u2019Hollander I et al (2007) Overexpression of mutant superoxide dismutase 1 causes a motor axonopathy in the zebrafish. Hum Mol Genet 16:2359\u20132365","journal-title":"Hum Mol Genet"},{"key":"741_CR129","doi-asserted-by":"publisher","first-page":"443","DOI":"10.1016\/0006-8993(79)90396-2","volume":"169","author":"SJ Lewkowicz","year":"1979","unstructured":"Lewkowicz SJ (1979) The relationship of Schwann cell migration in vitro to injury, using normal, wobbler, and dystrophic mice. Brain Res 169:443\u2013454","journal-title":"Brain Res"},{"key":"741_CR130","doi-asserted-by":"publisher","first-page":"147","DOI":"10.1016\/j.expneurol.2012.06.011","volume":"237","author":"B Liao","year":"2012","unstructured":"Liao B, Zhao W, Beers DR, Henkel JS, Appel SH (2012) Transformation from a neuroprotective to a neurotoxic microglial phenotype in a mouse model of ALS. Exp Neurol 237:147\u2013152","journal-title":"Exp Neurol"},{"key":"741_CR131","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1016\/j.yexcr.2005.01.022","volume":"306","author":"H Liewen","year":"2005","unstructured":"Liewen H, Meinhold-Heerlein I, Oliveira V, Schwarzenbacher R, Luo G et al (2005) Characterization of the human GARP (Golgi associated retrograde protein) complex. Exp Cell Res 306:24\u201334","journal-title":"Exp Cell Res"},{"key":"741_CR132","doi-asserted-by":"publisher","first-page":"459","DOI":"10.1097\/00005072-199705000-00001","volume":"56","author":"F Lisovoski","year":"1997","unstructured":"Lisovoski F, Blot S, Lacombe C, Bellier JP, Dreyfus PA, Junier MP (1997) Transforming growth factor alpha expression as a response of murine motor neurons to axonal injury and mutation-induced degeneration. J Neuropathol Exp Neurol 56:459\u2013471","journal-title":"J Neuropathol Exp Neurol"},{"key":"741_CR133","doi-asserted-by":"publisher","first-page":"e13250","DOI":"10.1371\/journal.pone.0013250","volume":"5","author":"L Liu-Yesucevitz","year":"2010","unstructured":"Liu-Yesucevitz L, Bilgutay A, Zhang YJ, Vanderweyde T, Citro A et al (2010) Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue. PLoS One 5:e13250","journal-title":"PLoS One"},{"key":"741_CR134","doi-asserted-by":"publisher","first-page":"995","DOI":"10.1016\/S1474-4422(10)70195-2","volume":"9","author":"IR Mackenzie","year":"2010","unstructured":"Mackenzie IR, Rademakers R, Neumann M (2010) TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia. Lancet Neurol 9:995\u20131007","journal-title":"Lancet Neurol"},{"key":"741_CR135","doi-asserted-by":"publisher","first-page":"443","DOI":"10.1038\/ng1016","volume":"32","author":"N Martin","year":"2002","unstructured":"Martin N, Jaubert J, Gounon P, Salido E, Haase G et al (2002) A missense mutation in Tbce causes progressive motor neuronopathy in mice. Nat Genet 32:443\u2013447","journal-title":"Nat Genet"},{"key":"741_CR136","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1038\/nature08971","volume":"465","author":"H Maruyama","year":"2010","unstructured":"Maruyama H, Morino H, Ito H, Izumi Y, Kato H et al (2010) Mutations of optineurin in amyotrophic lateral sclerosis. Nature 465:223\u2013226","journal-title":"Nature"},{"key":"741_CR137","doi-asserted-by":"publisher","first-page":"29626","DOI":"10.1074\/jbc.M203065200","volume":"277","author":"M Mattiazzi","year":"2002","unstructured":"Mattiazzi M, D\u2019Aurelio M, Gajewski CD, Martushova K, Kiaei M et al (2002) Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice. J Biol Chem 277:29626\u201329633","journal-title":"J Biol Chem"},{"key":"741_CR138","doi-asserted-by":"publisher","first-page":"361","DOI":"10.1111\/j.1748-1716.2010.02188.x","volume":"200","author":"CF Meehan","year":"2010","unstructured":"Meehan CF, Moldovan M, Marklund SL, Graffmo KS, Nielsen JB, Hultborn H (2010) Intrinsic properties of lumbar motor neurones in the adult G127insTGGG superoxide dismutase-1 mutant mouse in vivo: evidence for increased persistent inward currents. Acta Physiol (Oxf) 200:361\u2013376","journal-title":"Acta Physiol (Oxf)"},{"key":"741_CR139","doi-asserted-by":"publisher","first-page":"141","DOI":"10.1080\/17482960801934403","volume":"9","author":"MH Meisler","year":"2008","unstructured":"Meisler MH, Russ C, Montgomery KT, Greenway M, Ennis S et al (2008) Evaluation of the Golgi trafficking protein VPS54 (wobbler) as a candidate for ALS. Amyotroph Lateral Scler 9:141\u2013148","journal-title":"Amyotroph Lateral Scler"},{"key":"741_CR140","doi-asserted-by":"publisher","first-page":"153","DOI":"10.2119\/2006-00045.Mennini","volume":"12","author":"T Mennini","year":"2006","unstructured":"Mennini T, De Paola M, Bigini P, Mastrotto C, Fumagalli E et al (2006) Nonhematopoietic erythropoietin derivatives prevent motoneuron degeneration in vitro and in vivo. Mol Med 12:153\u2013160","journal-title":"Mol Med"},{"key":"741_CR141","doi-asserted-by":"publisher","first-page":"2791","DOI":"10.1242\/jcs.01130","volume":"117","author":"KE Miller","year":"2004","unstructured":"Miller KE, Sheetz MP (2004) Axonal mitochondrial transport and potential are correlated. J Cell Sci 117:2791\u20132804","journal-title":"J Cell Sci"},{"key":"741_CR142","doi-asserted-by":"publisher","first-page":"2031","DOI":"10.1016\/S0140-6736(07)60944-1","volume":"369","author":"JD Mitchell","year":"2007","unstructured":"Mitchell JD, Borasio GD (2007) Amyotrophic lateral sclerosis. Lancet 369:2031\u20132041","journal-title":"Lancet"},{"key":"741_CR143","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1007\/s00401-012-1043-z","volume":"125","author":"JC Mitchell","year":"2013","unstructured":"Mitchell JC, McGoldrick P, Vance C, Hortobagyi T, Sreedharan J et al (2013) Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion. Acta Neuropathol 125:273\u2013288","journal-title":"Acta Neuropathol"},{"issue":"Pt 4","key":"741_CR144","doi-asserted-by":"publisher","first-page":"811","DOI":"10.1093\/brain\/105.4.811","volume":"105","author":"H Mitsumoto","year":"1982","unstructured":"Mitsumoto H, Bradley WG (1982) Murine motor neuron disease (the wobbler mouse): degeneration and regeneration of the lower motor neuron. Brain 105(Pt 4):811\u2013834","journal-title":"Brain"},{"key":"741_CR145","doi-asserted-by":"publisher","first-page":"36","DOI":"10.1002\/ana.410190108","volume":"19","author":"H Mitsumoto","year":"1986","unstructured":"Mitsumoto H, Gambetti P (1986) Impaired slow axonal transport in wobbler mouse motor neuron disease. Ann Neurol 19:36\u201343","journal-title":"Ann Neurol"},{"key":"741_CR146","doi-asserted-by":"publisher","first-page":"121","DOI":"10.1002\/mus.880130206","volume":"13","author":"H Mitsumoto","year":"1990","unstructured":"Mitsumoto H, Ferut AL, Kurahashi K, McQuarrie IG (1990) Impairment of retrograde axonal transport in wobbler mouse motor neuron disease. Muscle Nerve 13:121\u2013126","journal-title":"Muscle Nerve"},{"key":"741_CR147","doi-asserted-by":"publisher","first-page":"542","DOI":"10.1002\/mus.880160517","volume":"16","author":"H Mitsumoto","year":"1993","unstructured":"Mitsumoto H, Kurahashi K, Jacob JM, McQuarrie IG (1993) Retardation of fast axonal transport in wobbler mice. Muscle Nerve 16:542\u2013547","journal-title":"Muscle Nerve"},{"key":"741_CR148","doi-asserted-by":"publisher","first-page":"1107","DOI":"10.1126\/science.8066451","volume":"265","author":"H Mitsumoto","year":"1994","unstructured":"Mitsumoto H, Ikeda K, Klinkosz B, Cedarbaum JM, Wong V, Lindsay RM (1994) Arrest of motor neuron disease in wobbler mice cotreated with CNTF and BDNF. Science 265:1107\u20131110","journal-title":"Science"},{"key":"741_CR149","doi-asserted-by":"publisher","first-page":"769","DOI":"10.1002\/mus.1068","volume":"24","author":"H Mitsumoto","year":"2001","unstructured":"Mitsumoto H, Klinkosz B, Pioro EP, Tsuzaka K, Ishiyama T et al (2001) Effects of cardiotrophin-1 (CT-1) in a mouse motor neuron disease. Muscle Nerve 24:769\u2013777","journal-title":"Muscle Nerve"},{"key":"741_CR150","doi-asserted-by":"publisher","first-page":"177","DOI":"10.1080\/17482960801933942","volume":"9","author":"H Mitsumoto","year":"2008","unstructured":"Mitsumoto H, Santella RM, Liu X, Bogdanov M, Zipprich J et al (2008) Oxidative stress biomarkers in sporadic ALS. Amyotroph Lateral Scler 9:177\u2013183","journal-title":"Amyotroph Lateral Scler"},{"key":"741_CR151","doi-asserted-by":"publisher","first-page":"e26066","DOI":"10.1371\/journal.pone.0026066","volume":"6","author":"A Moumen","year":"2011","unstructured":"Moumen A, Virard I, Raoul C (2011) Accumulation of wildtype and ALS-linked mutated VAPB impairs activity of the proteasome. PLoS One 6:e26066","journal-title":"PLoS One"},{"key":"741_CR152","doi-asserted-by":"publisher","first-page":"423","DOI":"10.1016\/0014-4886(80)90241-1","volume":"67","author":"T Murakami","year":"1980","unstructured":"Murakami T, Mastaglia FL, Bradley WG (1980) Reduced protein synthesis in spinal anterior horn neurons in wobbler mouse mutant. Exp Neurol 67:423\u2013432","journal-title":"Exp Neurol"},{"key":"741_CR153","doi-asserted-by":"publisher","first-page":"407","DOI":"10.1002\/mus.880040509","volume":"4","author":"T Murakami","year":"1981","unstructured":"Murakami T, Mastaglia FL, Mann DM, Bradley WG (1981) Abnormal RNA metabolism in spinal motor neurons in the wobbler mouse. Muscle Nerve 4:407\u2013412","journal-title":"Muscle Nerve"},{"key":"741_CR154","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 et al (2007) Astrocytes expressing ALS-linked mutated SOD1 release factors selectively toxic to motor neurons. Nat Neurosci 10:615\u2013622","journal-title":"Nat Neurosci"},{"key":"741_CR155","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 et al (2006) Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314:130\u2013133","journal-title":"Science"},{"key":"741_CR156","doi-asserted-by":"publisher","first-page":"625","DOI":"10.1093\/cercor\/bhq134","volume":"21","author":"J Nieto-Gonzalez","year":"2011","unstructured":"Nieto-Gonzalez J, Moser JM, Laurtizen M, Schmitt-John T, Jensen K (2011) Reduced GABAergic inhibition underlies cortical hyperexcitability in the wobbler mouse model of ALS. Cereb Cortex 21:625\u2013635","journal-title":"Cereb Cortex"},{"key":"741_CR157","doi-asserted-by":"publisher","first-page":"822","DOI":"10.1086\/425287","volume":"75","author":"AL Nishimura","year":"2004","unstructured":"Nishimura AL, Mitne-Neto M, Silva HC, Richieri-Costa A, Middleton S et al (2004) A mutation in the vesicle-trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis. Am J Hum Genet 75:822\u2013831","journal-title":"Am J Hum Genet"},{"key":"741_CR158","doi-asserted-by":"publisher","first-page":"52","DOI":"10.4161\/spmg.1.1.14698","volume":"1","author":"C Paiardi","year":"2011","unstructured":"Paiardi C, Pasini ME, Gioria M, Berruti G (2011) Failure of acrosome formation and globozoospermia in the wobbler mouse, a Vps54 spontaneous recessive mutant. Spermatogenesis 1:52\u201362","journal-title":"Spermatogenesis"},{"key":"741_CR159","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1186\/1471-2202-12-24","volume":"12","author":"R Palmisano","year":"2011","unstructured":"Palmisano R, Golfi P, Heimann P, Shaw CE, Troakes C et al (2011) Endosomal accumulation of APP in wobbler motor neurons reflects impaired vesicle trafficking: implications for human motor neuron disease pathologies. BMC Neuroscience 12:24","journal-title":"BMC Neuroscience"},{"key":"741_CR160","doi-asserted-by":"publisher","first-page":"R844","DOI":"10.1152\/ajpregu.00528.2010","volume":"300","author":"AV Panov","year":"2011","unstructured":"Panov AV, Kubalik N, Zinchenko N, Ridings DM, Radoff DA et al (2011) Metabolic and functional differences between brain and spinal cord mitochondria underlie different predisposition to pathology. Am J Physiol Regul Integr Comp Physiol 300:R844\u2013R854","journal-title":"Am J Physiol Regul Integr Comp Physiol"},{"key":"741_CR161","doi-asserted-by":"publisher","first-page":"493","DOI":"10.1016\/j.nbd.2009.10.005","volume":"37","author":"D Papadimitriou","year":"2010","unstructured":"Papadimitriou D, Le Verche V, Jacquier A, Ikiz B, Przedborski S, Re DB (2010) Inflammation in ALS and SMA: sorting out the good from the evil. Neurobiol Dis 37:493\u2013502","journal-title":"Neurobiol Dis"},{"key":"741_CR162","doi-asserted-by":"publisher","first-page":"1074","DOI":"10.1212\/01.wnl.0000231510.89311.8b","volume":"67","author":"N Parkinson","year":"2006","unstructured":"Parkinson N, Ince PG, Smith MO, Highley R, Skibinski G et al (2006) ALS phenotypes with mutations in CHMP2B (charged multivesicular body protein 2B). Neurology 67:1074\u20131077","journal-title":"Neurology"},{"key":"741_CR163","doi-asserted-by":"publisher","first-page":"5251","DOI":"10.1128\/MCB.00495-09","volume":"29","author":"FJ Perez-Victoria","year":"2009","unstructured":"Perez-Victoria FJ, Bonifacino JS (2009) Dual roles of the mammalian GARP complex in tethering and SNARE complex assembly at the trans-golgi network. Mol Cell Biol 29:5251\u20135263","journal-title":"Mol Cell Biol"},{"key":"741_CR164","doi-asserted-by":"publisher","first-page":"2350","DOI":"10.1091\/mbc.E07-11-1189","volume":"19","author":"FJ Perez-Victoria","year":"2008","unstructured":"Perez-Victoria FJ, Mardones GA, Bonifacino JS (2008) Requirement of the human GARP complex for mannose 6-phosphate-receptor-dependent sorting of cathepsin D to Lysosomes. Mol Biol Cell 19:2350\u20132362","journal-title":"Mol Biol Cell"},{"key":"741_CR165","doi-asserted-by":"publisher","first-page":"12860","DOI":"10.1073\/pnas.1004756107","volume":"107","author":"FJ Perez-Victoria","year":"2010","unstructured":"Perez-Victoria FJ, Abascal-Palacios G, Tascon I, Kajava A, Magadan JG et al (2010) Structural basis for the wobbler mouse neurodegenerative disorder caused by mutation in the Vps54 subunit of the GARP complex. Proc Natl Acad Sci USA 107:12860\u201312865","journal-title":"Proc Natl Acad Sci USA"},{"key":"741_CR166","doi-asserted-by":"publisher","first-page":"267","DOI":"10.1016\/0169-328X(95)00344-R","volume":"38","author":"R Pernas-Alonso","year":"1996","unstructured":"Pernas-Alonso R, Schaffner AE, Perrone-Capano C, Orlando A, Morelli F et al (1996) Early upregulation of medium neurofilament gene expression in developing spinal cord of the wobbler mouse mutant. Brain Res Mol Brain Res 38:267\u2013275","journal-title":"Brain Res Mol Brain Res"},{"key":"741_CR167","doi-asserted-by":"publisher","first-page":"581","DOI":"10.1006\/nbdi.2001.0403","volume":"8","author":"R Pernas-Alonso","year":"2001","unstructured":"Pernas-Alonso R, Perrone-Capano C, Volpicelli F, di Porzio U (2001) Regionalized neurofilament accumulation and motoneuron degeneration are linked phenotypes in wobbler neuromuscular disease. Neurobiol Dis 8:581\u2013589","journal-title":"Neurobiol Dis"},{"key":"741_CR168","doi-asserted-by":"publisher","first-page":"253","DOI":"10.1016\/S1474-4422(11)70015-1","volume":"10","author":"T Philips","year":"2011","unstructured":"Philips T, Robberecht W (2011) Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease. Lancet Neurol 10:253\u2013263","journal-title":"Lancet Neurol"},{"key":"741_CR169","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1007\/BF01188437","volume":"19","author":"MM Pollin","year":"1990","unstructured":"Pollin MM, McHanwell S, Slater CR (1990) Loss of motor neurons from the median nerve motor nucleus of the mutant mouse \u2018wobbler\u2019. J Neurocytol 19:29\u201338","journal-title":"J Neurocytol"},{"key":"741_CR170","doi-asserted-by":"publisher","first-page":"269","DOI":"10.1038\/ng1196-269","volume":"14","author":"SM Pulst","year":"1996","unstructured":"Pulst SM, Nechiporuk A, Nechiporuk T, Gispert S, Chen XN et al (1996) Moderate expansion of a normally biallelic trinucleotide repeat in spinocerebellar ataxia type 2. Nat Genet 14:269\u2013276","journal-title":"Nat Genet"},{"key":"741_CR171","doi-asserted-by":"publisher","first-page":"1859","DOI":"10.1091\/mbc.E05-11-1002","volume":"17","author":"NR Quenneville","year":"2006","unstructured":"Quenneville NR, Chao TY, McCaffery JM, Conibear E (2006) Domains within the GARP subunit Vps54 confer separate functions in complex assembly and early endosome recognition. Mol Biol Cell 17:1859\u20131870","journal-title":"Mol Biol Cell"},{"key":"741_CR172","doi-asserted-by":"publisher","first-page":"652","DOI":"10.1242\/dmm.005538","volume":"3","author":"T Ramesh","year":"2010","unstructured":"Ramesh T, Lyon AN, Pineda RH, Wang C, Janssen PM et al (2010) A genetic model of amyotrophic lateral sclerosis in zebrafish displays phenotypic hallmarks of motoneuron disease. Dis Model Mech 3:652\u2013662","journal-title":"Dis Model Mech"},{"key":"741_CR173","doi-asserted-by":"publisher","first-page":"3411","DOI":"10.1097\/00001756-199911080-00028","volume":"10","author":"S Rathke-Hartlieb","year":"1999","unstructured":"Rathke-Hartlieb S, Schmidt VC, Jockusch H, Schmitt-John T, Bartsch JW (1999) Spatiotemporal progression of neurodegeneration and glia activation in the wobbler neuropathy of the mouse. Neuroreport 10:3411\u20133416","journal-title":"Neuroreport"},{"key":"741_CR174","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, Majounie E, Waite A, Simon-Sanchez J, Rollinson S 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":"741_CR175","doi-asserted-by":"publisher","first-page":"893","DOI":"10.1007\/s003359900890","volume":"9","author":"K Resch","year":"1998","unstructured":"Resch K, Korthaus D, Wedemeyer N, Lengeling A, Ronsiek M et al (1998) Homology between human chromosome 2p13.3 and the wobbler critical region on mouse chromosome 11: comparative high-resolution mapping of STS and EST loci on YAC\/BAC contigs. Mamm Genome 9:893\u2013898","journal-title":"Mamm Genome"},{"key":"741_CR176","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1038\/364362c0","volume":"364","author":"DR Rosen","year":"1993","unstructured":"Rosen DR (1993) Mutations in Cu\/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 364:362","journal-title":"Nature"},{"key":"741_CR177","doi-asserted-by":"publisher","first-page":"1464","DOI":"10.1056\/NEJM199205283262204","volume":"326","author":"JD Rothstein","year":"1992","unstructured":"Rothstein JD, Martin LJ, Kuncl RW (1992) Decreased glutamate transport by the brain and spinal cord in amyotrophic lateral sclerosis. N Engl J Med 326:1464\u20131468","journal-title":"N Engl J Med"},{"key":"741_CR178","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":"741_CR179","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 et al (2005) Beta-lactam antibiotics offer neuroprotection by increasing glutamate transporter expression. Nature 433:73\u201377","journal-title":"Nature"},{"key":"741_CR180","doi-asserted-by":"publisher","first-page":"349","DOI":"10.1016\/j.nbd.2004.07.003","volume":"17","author":"B Santoro","year":"2004","unstructured":"Santoro B, Bigini P, Levandis G, Nobile V, Biggiogera M et al (2004) Evidence for chronic mitochondrial impairment in the cervical spinal cord of a murine model of motor neuron disease. Neurobiol Dis 17:349\u2013357","journal-title":"Neurobiol Dis"},{"key":"741_CR181","doi-asserted-by":"publisher","first-page":"397","DOI":"10.1086\/377158","volume":"73","author":"PC Sapp","year":"2003","unstructured":"Sapp PC, Hosler BA, McKenna-Yasek D, Chin W, Gann A et al (2003) Identification of two novel loci for dominantly inherited familial amyotrophic lateral sclerosis. Am J Hum Genet 73:397\u2013403","journal-title":"Am J Hum Genet"},{"key":"741_CR182","doi-asserted-by":"publisher","first-page":"241","DOI":"10.1002\/glia.20210","volume":"51","author":"SA Sargsyan","year":"2005","unstructured":"Sargsyan SA, Monk PN, Shaw PJ (2005) Microglia as potential contributors to motor neuron injury in amyotrophic lateral sclerosis. Glia 51:241\u2013253","journal-title":"Glia"},{"key":"741_CR183","doi-asserted-by":"publisher","first-page":"10","DOI":"10.1097\/nen.0b013e31802c396b","volume":"66","author":"S Sasaki","year":"2007","unstructured":"Sasaki S, Iwata M (2007) Mitochondrial alterations in the spinal cord of patients with sporadic amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 66:10\u201316","journal-title":"J Neuropathol Exp Neurol"},{"key":"741_CR184","doi-asserted-by":"publisher","first-page":"627","DOI":"10.1038\/nn.2297","volume":"12","author":"S Saxena","year":"2009","unstructured":"Saxena S, Cabuy E, Caroni P (2009) A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice. Nat Neurosci 12:627\u2013636","journal-title":"Nat Neurosci"},{"key":"741_CR185","doi-asserted-by":"publisher","first-page":"1213","DOI":"10.1038\/ng1661","volume":"37","author":"T Schmitt-John","year":"2005","unstructured":"Schmitt-John T, Drepper C, Mussmann A, Hahn P, Kuhlmann M et al (2005) Mutation of Vps54 causes motor neuron disease and defective spermiogenesis in the wobbler mouse. Nat Genet 37:1213\u20131215","journal-title":"Nat Genet"},{"key":"741_CR186","doi-asserted-by":"publisher","first-page":"16325","DOI":"10.1073\/pnas.1003459107","volume":"107","author":"X Shan","year":"2010","unstructured":"Shan X, Chiang PM, Price DL, Wong PC (2010) Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice. Proc Natl Acad Sci USA 107:16325\u201316330","journal-title":"Proc Natl Acad Sci USA"},{"key":"741_CR187","doi-asserted-by":"publisher","first-page":"209","DOI":"10.1006\/neur.1995.0026","volume":"4","author":"PJ Shaw","year":"1995","unstructured":"Shaw PJ, Forrest V, Ince PG, Richardson JP, Wastell HJ (1995a) CSF and plasma amino acid levels in motor neuron disease: elevation of CSF glutamate in a subset of patients. Neurodegeneration 4:209\u2013216","journal-title":"Neurodegeneration"},{"key":"741_CR188","doi-asserted-by":"publisher","first-page":"691","DOI":"10.1002\/ana.410380424","volume":"38","author":"PJ Shaw","year":"1995","unstructured":"Shaw PJ, Ince PG, Falkous G, Mantle D (1995b) Oxidative damage to protein in sporadic motor neuron disease spinal cord. Ann Neurol 38:691\u2013695","journal-title":"Ann Neurol"},{"key":"741_CR189","doi-asserted-by":"publisher","first-page":"149","DOI":"10.1016\/0304-3940(94)90956-3","volume":"179","author":"N Shibata","year":"1994","unstructured":"Shibata N, Hirano A, Kobayashi M, Sasaki S, Kato T et al (1994) Cu\/Zn superoxide dismutase-like immunoreactivity in Lewy body-like inclusions of sporadic amyotrophic lateral sclerosis. Neurosci Lett 179:149\u2013152","journal-title":"Neurosci Lett"},{"key":"741_CR190","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 et al (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":"741_CR191","doi-asserted-by":"publisher","first-page":"1758","DOI":"10.1212\/WNL.62.10.1758","volume":"62","author":"EP Simpson","year":"2004","unstructured":"Simpson EP, Henry YK, Henkel JS, Smith RG, Appel SH (2004) Increased lipid peroxidation in sera of ALS patients: a potential biomarker of disease burden. Neurology 62:1758\u20131765","journal-title":"Neurology"},{"key":"741_CR192","doi-asserted-by":"publisher","first-page":"696","DOI":"10.1002\/ana.410440419","volume":"44","author":"RG Smith","year":"1998","unstructured":"Smith RG, Henry YK, Mattson MP, Appel SH (1998) Presence of 4-hydroxynonenal in cerebrospinal fluid of patients with sporadic amyotrophic lateral sclerosis. Ann Neurol 44:696\u2013699","journal-title":"Ann Neurol"},{"key":"741_CR193","doi-asserted-by":"publisher","first-page":"402","DOI":"10.1007\/BF00308716","volume":"79","author":"G Sobue","year":"1990","unstructured":"Sobue G, Hashizume Y, Yasuda T, Mukai E, Kumagai T et al (1990) Phosphorylated high molecular weight neurofilament protein in lower motor neurons in amyotrophic lateral sclerosis and other neurodegenerative diseases involving ventral horn cells. Acta Neuropathol 79:402\u2013408","journal-title":"Acta Neuropathol"},{"key":"741_CR194","doi-asserted-by":"publisher","first-page":"340","DOI":"10.1016\/j.nbd.2003.11.012","volume":"15","author":"A Spalloni","year":"2004","unstructured":"Spalloni A, Albo F, Ferrari F, Mercuri N, Bernardi G et al (2004) Cu\/Zn-superoxide dismutase (GLY93\u00a0-->\u00a0ALA) mutation alters AMPA receptor subunit expression and function and potentiates kainate-mediated toxicity in motor neurons in culture. Neurobiol Dis 15:340\u2013350","journal-title":"Neurobiol Dis"},{"key":"741_CR195","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 et al (2008) TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science 319:1668\u20131672","journal-title":"Science"},{"key":"741_CR196","doi-asserted-by":"publisher","first-page":"595","DOI":"10.1016\/j.bbrc.2011.02.099","volume":"406","author":"L Staunton","year":"2011","unstructured":"Staunton L, Jockusch H, Ohlendieck K (2011) Proteomic analysis of muscle affected by motor neuron degeneration: the wobbler mouse model of amyotrophic lateral sclerosis. Biochem Biophys Res Commun 406:595\u2013600","journal-title":"Biochem Biophys Res Commun"},{"key":"741_CR197","doi-asserted-by":"publisher","first-page":"301","DOI":"10.1038\/nn823","volume":"5","author":"JR Subramaniam","year":"2002","unstructured":"Subramaniam JR, Lyons WE, Liu J, Bartnikas TB, Rothstein J et al (2002) Mutant SOD1 causes motor neuron disease independent of copper chaperone-mediated copper loading. Nat Neurosci 5:301\u2013307","journal-title":"Nat Neurosci"},{"issue":"5","key":"741_CR198","doi-asserted-by":"publisher","first-page":"1363","DOI":"10.1016\/j.celrep.2012.10.004","volume":"2","author":"M Sugimoto","year":"2012","unstructured":"Sugimoto M, Kondo M, Hirose M, Suzuki M, Mekada K et al (2012) Molecular identification of t(w5): Vps52 promotes pluripotential cell differentiation through cell\u2013cell interactions. Cell Rep 2:1363\u20131374","journal-title":"Cell Rep"},{"key":"741_CR199","doi-asserted-by":"publisher","first-page":"524","DOI":"10.1002\/cne.20130","volume":"474","author":"PG Sullivan","year":"2004","unstructured":"Sullivan PG, Rabchevsky AG, Keller JN, Lovell M, Sodhi A et al (2004) Intrinsic differences in brain and spinal cord mitochondria: implication for therapeutic interventions. J Comp Neurol 474:524\u2013534","journal-title":"J Comp Neurol"},{"key":"741_CR200","doi-asserted-by":"publisher","first-page":"973","DOI":"10.1111\/j.1471-4159.2008.05857.x","volume":"108","author":"H Suzuki","year":"2009","unstructured":"Suzuki H, Kanekura K, Levine TP, Kohno K, Olkkonen VM et al (2009) ALS-linked P56S-VAPB, an aggregated loss-of-function mutant of VAPB, predisposes motor neurons to ER stress-related death by inducing aggregation of co-expressed wild-type VAPB. J Neurochem 108:973\u2013985","journal-title":"J Neurochem"},{"key":"741_CR201","doi-asserted-by":"publisher","first-page":"535","DOI":"10.1007\/s00401-007-0206-9","volume":"113","author":"CF Tan","year":"2007","unstructured":"Tan CF, Eguchi H, Tagawa A, Onodera O, Iwasaki T et al (2007) TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation. Acta Neuropathol 113:535\u2013542","journal-title":"Acta Neuropathol"},{"key":"741_CR202","doi-asserted-by":"publisher","first-page":"353","DOI":"10.1016\/0006-8993(94)91805-8","volume":"650","author":"M Tomiyama","year":"1994","unstructured":"Tomiyama M, Kannari K, Nunomura J, Oyama Y, Takebe K, Matsunaga M (1994) Quantitative autoradiographic distribution of glutamate receptors in the cervical segment of the spinal cord of the wobbler mouse. Brain Res 650:353\u2013357","journal-title":"Brain Res"},{"key":"741_CR203","doi-asserted-by":"publisher","first-page":"134","DOI":"10.1002\/jnr.20715","volume":"83","author":"M Tortarolo","year":"2006","unstructured":"Tortarolo M, Grignaschi G, Calvaresi N, Zennaro E, Spaltro G et al (2006) Glutamate AMPA receptors change in motor neurons of SOD1G93A transgenic mice and their inhibition by a noncompetitive antagonist ameliorates the progression of amytrophic lateral sclerosis-like disease. J Neurosci Res 83:134\u2013146","journal-title":"J Neurosci Res"},{"key":"741_CR204","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1186\/1750-1326-4-31","volume":"4","author":"YRLB Tovar","year":"2009","unstructured":"Tovar YRLB, Santa-Cruz LD, Tapia R (2009) Experimental models for the study of neurodegeneration in amyotrophic lateral sclerosis. Mol Neurodegener 4:31","journal-title":"Mol Neurodegener"},{"key":"741_CR205","doi-asserted-by":"publisher","first-page":"474","DOI":"10.1002\/mus.1029","volume":"24","author":"K Tsuzaka","year":"2001","unstructured":"Tsuzaka K, Ishiyama T, Pioro EP, Mitsumoto H (2001) Role of brain-derived neurotrophic factor in wobbler mouse motor neuron disease. Muscle Nerve 24:474\u2013480","journal-title":"Muscle Nerve"},{"key":"741_CR206","doi-asserted-by":"publisher","first-page":"565","DOI":"10.1016\/S0306-4522(99)00095-0","volume":"93","author":"L Vergani","year":"1999","unstructured":"Vergani L, Losa M, Lesma E, Di Giulio AM, Torsello A et al (1999) Glycosaminoglycans boost insulin-like growth factor-I-promoted neuroprotection: blockade of motor neuron death in the wobbler mouse. Neuroscience 93:565\u2013572","journal-title":"Neuroscience"},{"key":"741_CR207","doi-asserted-by":"publisher","first-page":"695","DOI":"10.1016\/j.nbd.2010.12.005","volume":"41","author":"K Vijayalakshmi","year":"2011","unstructured":"Vijayalakshmi K, Alladi PA, Ghosh S, Prasanna VK, Sagar BC et al (2011) Evidence of endoplasmic reticular stress in the spinal motor neurons exposed to CSF from sporadic amyotrophic lateral sclerosis patients. Neurobiol Dis 41:695\u2013705","journal-title":"Neurobiol Dis"},{"key":"741_CR208","doi-asserted-by":"publisher","first-page":"1540","DOI":"10.1093\/brain\/awn071","volume":"131","author":"S Vucic","year":"2008","unstructured":"Vucic S, Nicholson GA, Kiernan MC (2008) Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosis. Brain 131:1540\u20131550","journal-title":"Brain"},{"key":"741_CR209","doi-asserted-by":"publisher","first-page":"177","DOI":"10.1016\/j.expneurol.2009.08.017","volume":"220","author":"S Vucic","year":"2009","unstructured":"Vucic S, Cheah BC, Kiernan MC (2009) Defining the mechanisms that underlie cortical hyperexcitability in amyotrophic lateral sclerosis. Exp Neurol 220:177\u2013182","journal-title":"Exp Neurol"},{"key":"741_CR210","doi-asserted-by":"publisher","first-page":"18809","DOI":"10.1073\/pnas.0908767106","volume":"106","author":"I Wegorzewska","year":"2009","unstructured":"Wegorzewska I, Bell S, Cairns NJ, Miller TM, Baloh RH (2009) TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci USA 106:18809\u201318814","journal-title":"Proc Natl Acad Sci USA"},{"key":"741_CR211","doi-asserted-by":"crossref","first-page":"2627","DOI":"10.1242\/jcs.115.13.2627","volume":"115","author":"JR Whyte","year":"2002","unstructured":"Whyte JR, Munro S (2002) Vesicle tethering complexes in membrane traffic. J Cell Sci 115:2627\u20132637","journal-title":"J Cell Sci"},{"key":"741_CR212","doi-asserted-by":"publisher","first-page":"616","DOI":"10.1046\/j.0022-3042.2001.00731.x","volume":"80","author":"FR Wiedemann","year":"2002","unstructured":"Wiedemann FR, Manfredi G, Mawrin C, Beal MF, Schon EA (2002) Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients. J Neurochem 80:616\u2013625","journal-title":"J Neurochem"},{"key":"741_CR213","doi-asserted-by":"publisher","first-page":"200","DOI":"10.1002\/ana.410420211","volume":"42","author":"TL Williams","year":"1997","unstructured":"Williams TL, Day NC, Ince PG, Kamboj RK, Shaw PJ (1997) Calcium-permeable alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid receptors: a molecular determinant of selective vulnerability in amyotrophic lateral sclerosis. Ann Neurol 42:200\u2013207","journal-title":"Ann Neurol"},{"key":"741_CR214","doi-asserted-by":"publisher","first-page":"50","DOI":"10.1038\/4553","volume":"2","author":"TL Williamson","year":"1999","unstructured":"Williamson TL, Cleveland DW (1999) Slowing of axonal transport is a very early event in the toxicity of ALS-linked SOD1 mutants to motor neurons. Nat Neurosci 2:50\u201356","journal-title":"Nat Neurosci"},{"key":"741_CR215","doi-asserted-by":"publisher","first-page":"3858","DOI":"10.1073\/pnas.0912417107","volume":"107","author":"H Wils","year":"2010","unstructured":"Wils H, Kleinberger G, Janssens J, Pereson S, Joris G et al (2010) TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration. Proc Natl Acad Sci USA 107:3858\u20133863","journal-title":"Proc Natl Acad Sci USA"},{"key":"741_CR216","doi-asserted-by":"publisher","first-page":"1105","DOI":"10.1016\/0896-6273(95)90259-7","volume":"14","author":"PC Wong","year":"1995","unstructured":"Wong PC, Pardo CA, Borchelt DR, Lee MK, Copeland NG et al (1995) An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria. Neuron 14:1105\u20131116","journal-title":"Neuron"},{"key":"741_CR217","doi-asserted-by":"publisher","first-page":"529","DOI":"10.1046\/j.0305-1846.2003.00518.x","volume":"29","author":"JD Wood","year":"2003","unstructured":"Wood JD, Beaujeux TP, Shaw PJ (2003) Protein aggregation in motor neurone disorders. Neuropathol Appl Neurobiol 29:529\u2013545","journal-title":"Neuropathol Appl Neurobiol"},{"key":"741_CR218","doi-asserted-by":"publisher","first-page":"252","DOI":"10.7861\/clinmedicine.10-3-252","volume":"10","author":"C Wood-Allum","year":"2010","unstructured":"Wood-Allum C, Shaw PJ (2010) Motor neurone disease: a practical update on diagnosis and management. Clin Med 10:252\u2013258","journal-title":"Clin Med"},{"key":"741_CR219","doi-asserted-by":"publisher","first-page":"499","DOI":"10.1038\/nature11280","volume":"488","author":"CH Wu","year":"2012","unstructured":"Wu CH, Fallini C, Ticozzi N, Keagle PJ, Sapp PC et al (2012) Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis. Nature 488:499\u2013503","journal-title":"Nature"},{"key":"741_CR220","doi-asserted-by":"publisher","first-page":"23","DOI":"10.1016\/0092-8674(93)90157-L","volume":"73","author":"Z Xu","year":"1993","unstructured":"Xu Z, Cork LC, Griffin JW, Cleveland DW (1993) Increased expression of neurofilament subunit NF-L produces morphological alterations that resemble the pathology of human motor neuron disease. Cell 73:23\u201333","journal-title":"Cell"},{"key":"741_CR221","doi-asserted-by":"publisher","first-page":"141","DOI":"10.1016\/S0304-3940(01)01575-0","volume":"300","author":"GP Xu","year":"2001","unstructured":"Xu GP, Dave KR, Moraes CT, Busto R, Sick TJ et al (2001) Dysfunctional mitochondrial respiration in the wobbler mouse brain. Neurosci Lett 300:141\u2013144","journal-title":"Neurosci Lett"},{"key":"741_CR222","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 et al (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":"741_CR223","doi-asserted-by":"publisher","first-page":"e1000887","DOI":"10.1371\/journal.pgen.1000887","volume":"6","author":"H Zhou","year":"2010","unstructured":"Zhou H, Huang C, Chen H, Wang D, Landel CP et al (2010) Transgenic rat model of neurodegeneration caused by mutation in the TDP gene. PLoS Genet 6:e1000887","journal-title":"PLoS Genet"},{"key":"741_CR224","doi-asserted-by":"publisher","first-page":"23432","DOI":"10.1074\/jbc.M111.237818","volume":"286","author":"YB Zhu","year":"2011","unstructured":"Zhu YB, Sheng ZH (2011) Increased axonal mitochondrial mobility does not slow amyotrophic lateral sclerosis (ALS)-like disease in mutant SOD1 mice. J Biol Chem 286:23432\u201323440","journal-title":"J Biol Chem"}],"container-title":["Molecular Genetics and Genomics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s00438-013-0741-0\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00438-013-0741-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00438-013-0741-0","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00438-013-0741-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,2,12]],"date-time":"2022-02-12T16:36:26Z","timestamp":1644683786000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00438-013-0741-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,3,29]]},"references-count":224,"journal-issue":{"issue":"5-6","published-print":{"date-parts":[[2013,6]]}},"alternative-id":["741"],"URL":"https:\/\/doi.org\/10.1007\/s00438-013-0741-0","relation":{},"ISSN":["1617-4615","1617-4623"],"issn-type":[{"value":"1617-4615","type":"print"},{"value":"1617-4623","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,3,29]]}}}