{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T19:24:39Z","timestamp":1787081079848,"version":"build-2736575974"},"reference-count":211,"publisher":"Frontiers Media SA","content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Cell. Neurosci."],"DOI":"10.3389\/fncel.2017.00126","type":"journal-article","created":{"date-parts":[[2017,5,4]],"date-time":"2017-05-04T00:32:53Z","timestamp":1493857973000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":16,"title":["Energy Homeostasis and Abnormal RNA Metabolism in Amyotrophic Lateral Sclerosis"],"prefix":"10.3389","volume":"11","author":[{"given":"Yu-Ju","family":"Liu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Po-Yi","family":"Tsai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yijuang","family":"Chern","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2017,5,4]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"332","DOI":"10.1016\/S1474-4422(15)00380-4","article-title":"Amyotrophic lateral sclerosis and frontotemporal dementia: distinct and overlapping changes in eating behaviour and metabolism","volume":"15","author":"Ahmed","year":"2016","journal-title":"Lancet Neurol."},{"key":"B2","doi-asserted-by":"publisher","first-page":"1182","DOI":"10.1016\/S1474-4422(16)30199-5","article-title":"Amyotrophic lateral sclerosis: moving towards a new classification system","volume":"15","author":"Al-Chalabi","year":"2016","journal-title":"Lancet Neurol."},{"key":"B3","doi-asserted-by":"publisher","first-page":"141","DOI":"10.1016\/j.tibs.2007.12.003","article-title":"Stress granules: the Tao of RNA triage","volume":"33","author":"Anderson","year":"2008","journal-title":"Trends Biochem. Sci."},{"key":"B4","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1186\/1471-2121-9-37","article-title":"The multifunctional FUS, EWS and TAF15 proto-oncoproteins show cell type-specific expression patterns and involvement in cell spreading and stress response","volume":"9","author":"Andersson","year":"2008","journal-title":"BMC Cell Biol."},{"key":"B5","doi-asserted-by":"publisher","first-page":"602","DOI":"10.1016\/j.bbrc.2006.10.093","article-title":"TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis","volume":"351","author":"Arai","year":"2006","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"B6","doi-asserted-by":"publisher","first-page":"423","DOI":"10.3389\/fncel.2015.00423","article-title":"Alterations in stress granule dynamics driven by TDP-43 and FUS: a link to pathological inclusions in ALS?","volume":"9","author":"Aulas","year":"2015","journal-title":"Front. Cell. Neurosci."},{"key":"B7","doi-asserted-by":"publisher","first-page":"259","DOI":"10.1007\/s00401-011-0850-y","article-title":"Cell stress induces TDP-43 pathological changes associated with ERK1\/2 dysfunction: implications in ALS","volume":"122","author":"Ayala","year":"2011","journal-title":"Acta Neuropathol."},{"key":"B8","doi-asserted-by":"publisher","first-page":"413","DOI":"10.1038\/nature02544","article-title":"VEGF delivery with retrogradely transported lentivector prolongs survival in a mouse ALS model","volume":"429","author":"Azzouz","year":"2004","journal-title":"Nature"},{"key":"B9","doi-asserted-by":"publisher","first-page":"2385","DOI":"10.1128\/MCB.00087-15","article-title":"RBM45 modulates the antioxidant response in amyotrophic lateral sclerosis through interactions with KEAP1","volume":"35","author":"Bakkar","year":"2015","journal-title":"Mol. Cell. Biol."},{"key":"B10","doi-asserted-by":"publisher","first-page":"3539","DOI":"10.1111\/j.1742-4658.2011.08256.x","article-title":"TDP-43: the relationship between protein aggregation and neurodegeneration in amyotrophic lateral sclerosis and frontotemporal lobar degeneration","volume":"278","author":"Baloh","year":"2011","journal-title":"FEBS J."},{"key":"B11","doi-asserted-by":"publisher","first-page":"e1002481","DOI":"10.1371\/journal.pgen.1002481","article-title":"A pathogenic mechanism in Huntington\u2019s disease involves small CAG-repeated RNAs with neurotoxic activity","volume":"8","author":"Ba\u00f1ez-Coronel","year":"2012","journal-title":"PLoS Genet."},{"key":"B12","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1186\/1750-1326-8-30","article-title":"Amyotrophic lateral sclerosis-linked FUS\/TLS alters stress granule assembly and dynamics","volume":"8","author":"Baron","year":"2013","journal-title":"Mol. Neurodegener."},{"key":"B13","doi-asserted-by":"publisher","first-page":"611","DOI":"10.1212\/WNL.0b013e3181ed9cde","article-title":"Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutations","volume":"75","author":"B\u00e4umer","year":"2010","journal-title":"Neurology"},{"key":"B14","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1016\/j.neulet.2004.12.071","article-title":"Delocalization of the multifunctional RNA splicing factor TLS\/FUS in hippocampal neurones: exclusion from the nucleus and accumulation in dendritic granules and spine heads","volume":"379","author":"Belly","year":"2005","journal-title":"Neurosci. Lett."},{"key":"B15","doi-asserted-by":"publisher","first-page":"23079","DOI":"10.1074\/jbc.M111.328757","article-title":"Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43)","volume":"287","author":"Bentmann","year":"2012","journal-title":"J. Biol. Chem."},{"key":"B16","doi-asserted-by":"publisher","first-page":"2247","DOI":"10.1111\/j.1460-9568.2010.07260.x","article-title":"The neurobiology of amyotrophic lateral sclerosis","volume":"31","author":"Bento-Abreu","year":"2010","journal-title":"Eur. J. Neurosci."},{"key":"B17","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1016\/s0969-9961(03)00043-3","article-title":"Mitochondrial dysfunction due to mutant copper\/zinc superoxide dismutase associated with amyotrophic lateral sclerosis is reversed by N-acetylcysteine","volume":"13","author":"Beretta","year":"2003","journal-title":"Neurobiol. Dis."},{"key":"B18","doi-asserted-by":"publisher","first-page":"765","DOI":"10.1093\/brain\/aws004","article-title":"Characterization of frontotemporal dementia and\/or amyotrophic lateral sclerosis associated with the GGGGCC repeat expansion in C9ORF72","volume":"135","author":"Boeve","year":"2012","journal-title":"Acta Neuropathol. Commun."},{"key":"B19","doi-asserted-by":"publisher","first-page":"20877","DOI":"10.1038\/srep20877","article-title":"Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS\/FTD","volume":"6","author":"Boeynaems","year":"","journal-title":"Sci. Rep."},{"key":"B20","doi-asserted-by":"publisher","first-page":"159","DOI":"10.1007\/s00401-016-1586-5","article-title":"Inside out: the role of nucleocytoplasmic transport in ALS and FTLD","volume":"132","author":"Boeynaems","year":"","journal-title":"Acta Neuropathol."},{"key":"B21","doi-asserted-by":"publisher","first-page":"787","DOI":"10.1002\/1531-8249(199911)46:5<787::AID-ANA17>3.0.CO;2-8","article-title":"Mitochondrial enzyme activity in amyotrophic lateral sclerosis: implications for the role of mitochondria in neuronal cell death","volume":"46","author":"Borthwick","year":"1999","journal-title":"Ann. Neurol."},{"key":"B22","doi-asserted-by":"publisher","first-page":"4160","DOI":"10.1093\/hmg\/ddq335","article-title":"Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granules","volume":"19","author":"Bosco","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"B23","doi-asserted-by":"publisher","first-page":"44441","DOI":"10.1074\/jbc.M111.237115","article-title":"Regulation of autophagy by neuropathological protein TDP-43","volume":"286","author":"Bose","year":"2011","journal-title":"J. Biol. Chem."},{"key":"B24","doi-asserted-by":"publisher","first-page":"385","DOI":"10.1016\/0092-8674(92)90154-5","article-title":"Molecular basis of myotonic dystrophy: expansion of a trinucleotide (CTG) repeat at the 3\u2032 end of a transcript encoding a protein kinase family member","volume":"69","author":"Brook","year":"1992","journal-title":"Cell"},{"key":"B25","doi-asserted-by":"publisher","first-page":"1851","DOI":"10.1126\/science.281.5384.1851","article-title":"Aggregation and motor neuron toxicity of an ALS-linked SOD1 mutant independent from wild-type SOD1","volume":"281","author":"Bruijn","year":"1998","journal-title":"Science"},{"key":"B26","doi-asserted-by":"publisher","first-page":"932","DOI":"10.1016\/j.molcel.2009.11.020","article-title":"Eukaryotic stress granules: the ins and outs of translation","volume":"36","author":"Buchan","year":"2009","journal-title":"Mol. Cell"},{"key":"B27","doi-asserted-by":"publisher","first-page":"2268","DOI":"10.1111\/j.1742-4658.2010.07643.x","article-title":"Nuclear factor TDP-43 can affect selected microRNA levels","volume":"277","author":"Buratti","year":"2010","journal-title":"FEBS J."},{"key":"B28","doi-asserted-by":"publisher","first-page":"232","DOI":"10.1016\/S1474-4422(12)70014-5","article-title":"Cognitive and clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat expansion: a population-based cohort study","volume":"11","author":"Byrne","year":"2012","journal-title":"Lancet Neurol."},{"key":"B29","doi-asserted-by":"publisher","first-page":"e1001288","DOI":"10.1371\/journal.pgen.1001288","article-title":"Pathogenic VCP\/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model","volume":"7","author":"Chang","year":"2011","journal-title":"PLoS Genet."},{"key":"B30","doi-asserted-by":"publisher","first-page":"16320","DOI":"10.1073\/pnas.1002176107","article-title":"Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism","volume":"107","author":"Chiang","year":"2010","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"B31","doi-asserted-by":"publisher","first-page":"1681","DOI":"10.1212\/WNL.0b013e3181c1df1e","article-title":"Lower serum lipid levels are related to respiratory impairment in patients with ALS","volume":"73","author":"Chio","year":"","journal-title":"Neurology"},{"key":"B33","doi-asserted-by":"publisher","first-page":"310","DOI":"10.3109\/17482960802566824","article-title":"Prognostic factors in ALS: a critical review","volume":"10","author":"Chio","year":"","journal-title":"Amyotroph. Lateral Scler."},{"key":"B32","doi-asserted-by":"publisher","first-page":"784","DOI":"10.1093\/brain\/awr366","article-title":"Clinical characteristics of patients with familial amyotrophic lateral sclerosis carrying the pathogenic GGGGCC hexanucleotide repeat expansion of C9ORF72","volume":"135","author":"Chio","year":"2012","journal-title":"Brain"},{"key":"B34","doi-asserted-by":"publisher","first-page":"310","DOI":"10.1111\/j.1471-4159.2005.03029.x","article-title":"CGS21680 attenuates symptoms of Huntington\u2019s disease in a transgenic mouse model","volume":"93","author":"Chou","year":"2005","journal-title":"J. Neurochem."},{"key":"B35","doi-asserted-by":"publisher","first-page":"180","DOI":"10.1002\/ana.23946","article-title":"Loss of function of C9orf72 causes motor deficits in a zebrafish model of amyotrophic lateral sclerosis","volume":"74","author":"Ciura","year":"2013","journal-title":"Ann. Neurol."},{"key":"B36","doi-asserted-by":"publisher","first-page":"717","DOI":"10.1007\/s00401-012-1045-x","article-title":"The RNA-binding motif 45 (RBM45) protein accumulates in inclusion bodies in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) patients","volume":"124","author":"Collins","year":"2012","journal-title":"Acta Neuropathol."},{"key":"B37","doi-asserted-by":"publisher","first-page":"1068","DOI":"10.1002\/pds.1643","article-title":"An evaluation of a data mining signal for amyotrophic lateral sclerosis and statins detected in FDA\u2019s spontaneous adverse event reporting system","volume":"17","author":"Colman","year":"2008","journal-title":"Pharmacoepidemiol. Drug Saf."},{"key":"B38","doi-asserted-by":"publisher","first-page":"15635","DOI":"10.1074\/jbc.M111.333450","article-title":"TDP-43 and FUS RNA-binding proteins bind distinct sets of cytoplasmic messenger RNAs and differently regulate their post-transcriptional fate in motoneuron-like cells","volume":"287","author":"Colombrita","year":"2012","journal-title":"J. Biol. Chem."},{"key":"B39","doi-asserted-by":"publisher","first-page":"1051","DOI":"10.1111\/j.1471-4159.2009.06383.x","article-title":"TDP-43 is recruited to stress granules in conditions of oxidative insult","volume":"111","author":"Colombrita","year":"2009","journal-title":"J. Neurochem."},{"key":"B40","doi-asserted-by":"publisher","first-page":"751","DOI":"10.5772\/30897","article-title":"Clinico-pathological features in amyotrophic lateral sclerosis with expansions in C9ORF72","volume":"135","author":"Cooper-Knock","year":"2012","journal-title":"Brain"},{"key":"B41","doi-asserted-by":"publisher","first-page":"e0140248","DOI":"10.1371\/journal.pone.0140248","article-title":"Inflammation induces TDP-43 mislocalization and aggregation","volume":"10","author":"Correia","year":"2015","journal-title":"PLoS One"},{"key":"B42","doi-asserted-by":"publisher","first-page":"558","DOI":"10.1111\/j.1432-1033.1995.tb20498.x","article-title":"5-aminoimidazole-4-carboxamide ribonucleoside. A specific method for activating AMP-activated protein kinase in intact cells?","volume":"229","author":"Corton","year":"1995","journal-title":"Eur. J. Biochem."},{"key":"B43","doi-asserted-by":"publisher","first-page":"217","DOI":"10.1016\/j.ceca.2011.02.004","article-title":"G37R SOD1 mutant alters mitochondrial complex I activity, Ca2+ uptake and ATP production","volume":"49","author":"Coussee","year":"2011","journal-title":"Cell Calcium"},{"key":"B44","doi-asserted-by":"publisher","first-page":"74","DOI":"10.1016\/j.nbd.2014.07.009","article-title":"Aggregate formation prevents dTDP-43 neurotoxicity in the Drosophila melanogaster eye","volume":"71","author":"Cragnaz","year":"2014","journal-title":"Neurobiol. Dis."},{"key":"B45","doi-asserted-by":"publisher","first-page":"849","DOI":"10.1001\/archneurol.2010.128","article-title":"Mitochondrial respiratory chain dysfunction in muscle from patients with amyotrophic lateral sclerosis","volume":"67","author":"Crugnola","year":"2010","journal-title":"Arch. Neurol."},{"key":"B46","doi-asserted-by":"publisher","first-page":"2063","DOI":"10.1002\/stem.2388","article-title":"C9orf72 hexanucleotide expansions are associated with altered endoplasmic reticulum calcium homeostasis and stress granule formation in induced pluripotent stem cell-derived neurons from patients with amyotrophic lateral sclerosis and frontotemporal dementia","volume":"34","author":"Dafinca","year":"2016","journal-title":"Stem Cells"},{"key":"B47","doi-asserted-by":"publisher","first-page":"477","DOI":"10.1038\/330477a0","article-title":"Selective dendritic transport of RNA in hippocampal neurons in culture","volume":"330","author":"Davis","year":"1987","journal-title":"Nature"},{"key":"B48","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1016\/j.neuron.2011.09.011","article-title":"Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS","volume":"72","author":"DeJesus-Hernandez","year":"2011","journal-title":"Neuron"},{"key":"B49","doi-asserted-by":"publisher","first-page":"e1005357","DOI":"10.1371\/journal.pgen.1005357","article-title":"FUS interacts with HSP60 to promote mitochondrial damage","volume":"11","author":"Deng","year":"2015","journal-title":"PLoS Genet."},{"key":"B50","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1007\/s00109-015-1276-0","article-title":"Nuclear-cytoplasmatic shuttling of proteins in control of cellular oxygen sensing","volume":"93","author":"Depping","year":"2015","journal-title":"J. Mol. Med."},{"key":"B53","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1093\/ajcn\/74.3.328","article-title":"Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis","volume":"74","author":"Desport","year":"2001","journal-title":"Am. J. Clin. Nutr."},{"key":"B52","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1080\/14660820050515386","article-title":"Nutritional assessment and survival in ALS patients","volume":"1","author":"Desport","year":"2000","journal-title":"Amyotroph. Lateral Scler Other Motor Neuron Disord."},{"key":"B54","doi-asserted-by":"publisher","first-page":"1059","DOI":"10.1212\/WNL.53.5.1059","article-title":"Nutritional status is a prognostic factor for survival in ALS patients","volume":"53","author":"Desport","year":"1999","journal-title":"Neurology"},{"key":"B51","doi-asserted-by":"publisher","first-page":"202","DOI":"10.1159\/000089626","article-title":"Hypermetabolism in ALS: correlations with clinical and paraclinical parameters","volume":"2","author":"Desport","year":"2005","journal-title":"Neurodegener. Dis."},{"key":"B56","doi-asserted-by":"publisher","first-page":"1098","DOI":"10.1128\/MCB.01279-10","article-title":"TDP-43 is directed to stress granules by sorbitol, a novel physiological osmotic and oxidative stressor","volume":"31","author":"Dewey","year":"2011","journal-title":"Mol. Cell. Biol."},{"key":"B55","doi-asserted-by":"publisher","first-page":"16","DOI":"10.1016\/j.brainres.2012.02.032","article-title":"TDP-43 aggregation in neurodegeneration: are stress granules the key?","volume":"1462","author":"Dewey","year":"2012","journal-title":"Brain Res."},{"key":"B57","doi-asserted-by":"publisher","first-page":"425","DOI":"10.1016\/j.cmet.2008.09.002","article-title":"Skeletal muscle is a primary target of SOD1G93A-mediated toxicity","volume":"8","author":"Dobrowolny","year":"2008","journal-title":"Cell Metab."},{"key":"B58","doi-asserted-by":"publisher","first-page":"2165","DOI":"10.1016\/j.cellsig.2008.05.007","article-title":"Signalling pathways regulating nucleo-cytoplasmic shuttling of the mRNA-binding protein HuR","volume":"20","author":"Doller","year":"2008","journal-title":"Cell. Signal."},{"key":"B59","doi-asserted-by":"publisher","first-page":"26758","DOI":"10.1038\/srep26758","article-title":"AMP-activated protein kinase modulates tau phosphorylation and tau pathology in vivo","volume":"6","author":"Domise","year":"2016","journal-title":"Sci. Rep."},{"key":"B60","doi-asserted-by":"publisher","first-page":"415","DOI":"10.1016\/j.neuron.2013.10.015","article-title":"RNA toxicity from the ALS\/FTD C9ORF72 expansion is mitigated by antisense intervention","volume":"80","author":"Donnelly","year":"2013","journal-title":"Neuron"},{"key":"B61","doi-asserted-by":"publisher","first-page":"339","DOI":"10.1016\/j.tins.2011.05.002","article-title":"TDP-43 and FUS: a nuclear affair","volume":"34","author":"Dormann","year":"2011","journal-title":"Trends Neurosci."},{"key":"B62","doi-asserted-by":"publisher","first-page":"2841","DOI":"10.1038\/emboj.2010.143","article-title":"ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import","volume":"29","author":"Dormann","year":"2010","journal-title":"EMBO J."},{"key":"B63","doi-asserted-by":"publisher","first-page":"613","DOI":"10.1007\/s00415-010-5805-z","article-title":"Patients with elevated triglyceride and cholesterol serum levels have a prolonged survival in amyotrophic lateral sclerosis","volume":"258","author":"Dorst","year":"2011","journal-title":"J. Neurol."},{"key":"B64","doi-asserted-by":"publisher","first-page":"336","DOI":"10.1111\/j.1365-2990.2011.01166.x","article-title":"Review: the role of mitochondria in the pathogenesis of amyotrophic lateral sclerosis","volume":"37","author":"Duffy","year":"2011","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"B67","doi-asserted-by":"publisher","first-page":"1004","DOI":"10.1212\/01.wnl.0000285080.70324.27","article-title":"Dyslipidemia is a protective factor in amyotrophic lateral sclerosis","volume":"70","author":"Dupuis","year":"2008","journal-title":"Neurology"},{"key":"B66","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1159\/000085063","article-title":"Mitochondria in amyotrophic lateral sclerosis: a trigger and a target","volume":"1","author":"Dupuis","year":"","journal-title":"Neurodegener. Dis."},{"key":"B65","doi-asserted-by":"publisher","first-page":"11159","DOI":"10.1073\/pnas.0402026101","article-title":"Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model","volume":"101","author":"Dupuis","year":"","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"B68","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1016\/S1474-4422(10)70224-6","article-title":"Energy metabolism in amyotrophic lateral sclerosis","volume":"10","author":"Dupuis","year":"2011","journal-title":"Lancet Neurol."},{"key":"B69","doi-asserted-by":"publisher","first-page":"623","DOI":"10.1002\/ana.10357","article-title":"Mitochondrial respiratory chain function in skeletal muscle of ALS patients","volume":"52","author":"Echaniz-Laguna","year":"2002","journal-title":"Ann. Neurol."},{"key":"B70","doi-asserted-by":"publisher","first-page":"515","DOI":"10.2165\/00002018-200730060-00005","article-title":"Statins, neuromuscular degenerative disease and an amyotrophic lateral sclerosis-like syndrome: an analysis of individual case safety reports from vigibase","volume":"30","author":"Edwards","year":"2007","journal-title":"Drug Saf."},{"key":"B71","doi-asserted-by":"publisher","first-page":"1586","DOI":"10.1016\/j.febslet.2009.04.019","article-title":"Depletion of TDP-43 affects Drosophila motoneurons terminal synapsis and locomotive behavior","volume":"583","author":"Feiguin","year":"2009","journal-title":"FEBS Lett."},{"key":"B72","doi-asserted-by":"publisher","first-page":"1571","DOI":"10.1194\/jlr.m700017-jlr200","article-title":"Increased peripheral lipid clearance in an animal model of amyotrophic lateral sclerosis","volume":"48","author":"Fergani","year":"2007","journal-title":"J. Lipid Res."},{"key":"B73","doi-asserted-by":"publisher","first-page":"4529","DOI":"10.1093\/hmg\/ddq383","article-title":"Glutaredoxin 2 prevents aggregation of mutant SOD1 in mitochondria and abolishes its toxicity","volume":"19","author":"Ferri","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"B74","doi-asserted-by":"publisher","first-page":"1104","DOI":"10.1021\/pr901076y","article-title":"Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery","volume":"9","author":"Freibaum","year":"2010","journal-title":"J. Proteome Res."},{"key":"B75","doi-asserted-by":"publisher","first-page":"129","DOI":"10.1038\/nature14974","article-title":"GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport","volume":"525","author":"Freibaum","year":"2015","journal-title":"Nature"},{"key":"B77","doi-asserted-by":"publisher","first-page":"587","DOI":"10.3410\/f.1009219.325127","article-title":"The RNA binding protein TLS is translocated to dendritic spines by mGluR5 activation and regulates spine morphology","volume":"15","author":"Fujii","year":"2005","journal-title":"Curr. Biol."},{"key":"B76","doi-asserted-by":"publisher","first-page":"5755","DOI":"10.1242\/jcs.02692","article-title":"TLS facilitates transport of mRNA encoding an actin-stabilizing protein to dendritic spines","volume":"118","author":"Fujii","year":"2005","journal-title":"J. Cell Sci."},{"key":"B78","doi-asserted-by":"publisher","first-page":"2323.e27","DOI":"10.1016\/j.neurobiolaging.2010.06.010","article-title":"Nuclear localization sequence of FUS and induction of stress granules by ALS mutants","volume":"32","author":"Gal","year":"2011","journal-title":"Neurobiol. Aging"},{"key":"B79","doi-asserted-by":"publisher","first-page":"54","DOI":"10.1016\/S1474-4422(11)70261-7","article-title":"A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study","volume":"11","author":"Gijselinck","year":"2012","journal-title":"Lancet Neurol."},{"key":"B80","doi-asserted-by":"publisher","first-page":"351","DOI":"10.1111\/j.1750-3639.2009.00284.x","article-title":"TDP-43 redistribution is an early event in sporadic amyotrophic lateral sclerosis","volume":"20","author":"Giordana","year":"2010","journal-title":"Brain Pathol."},{"key":"B81","doi-asserted-by":"publisher","first-page":"649","DOI":"10.2165\/00002018-200932080-00004","article-title":"Amyotrophic lateral sclerosis-like conditions in possible association with cholesterol-lowering drugs: an analysis of patient reports to the university of California, San Diego (UCSD) statin effects study","volume":"32","author":"Golomb","year":"2009","journal-title":"Drug Saf."},{"key":"B82","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1146\/annurev.cellbio.15.1.607","article-title":"Transport between the cell nucleus and the cytoplasm","volume":"15","author":"G\u00f6rlich","year":"1999","journal-title":"Annu. Rev. Cell Dev. Biol."},{"key":"B83","doi-asserted-by":"publisher","first-page":"348","DOI":"10.1159\/000017460","article-title":"Role of mitochondria in neuronal apoptosis","volume":"22","author":"Gorman","year":"2000","journal-title":"Dev. Neurosci."},{"key":"B84","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1038\/nature03120","article-title":"The microprocessor complex mediates the genesis of microRNAs","volume":"432","author":"Gregory","year":"2004","journal-title":"Nature"},{"key":"B85","doi-asserted-by":"publisher","first-page":"429","DOI":"10.1111\/j.1600-0404.1991.tb04983.x","article-title":"Motor neuron disease and dementia reported among 13 members of a single family","volume":"84","author":"Gunnarsson","year":"1991","journal-title":"Acta Neurol. Scand."},{"key":"B86","doi-asserted-by":"publisher","first-page":"4838","DOI":"10.1074\/jbc.273.9.4838","article-title":"The transcription factor Spi-1\/PU.1 interacts with the potential splicing factor TLS","volume":"273","author":"Hallier","year":"1998","journal-title":"J. Biol. Chem."},{"key":"B87","doi-asserted-by":"publisher","first-page":"S7","DOI":"10.1038\/ijo.2008.116","article-title":"AMPK: a key regulator of energy balance in the single cell and the whole organism","volume":"32","author":"Hardie","year":"2008","journal-title":"Int. J. Obes. (Lond)"},{"key":"B88","doi-asserted-by":"publisher","first-page":"1428","DOI":"10.1038\/nsmb.2163","article-title":"RNA targets of wild-type and mutant FET family proteins","volume":"18","author":"Hoell","year":"2011","journal-title":"Nat. Struct. Mol. Biol."},{"key":"B89","doi-asserted-by":"publisher","first-page":"1069","DOI":"10.1111\/j.1750-3639.2010.00413.x","article-title":"Extensive FUS-immunoreactive pathology in juvenile amyotrophic lateral sclerosis with basophilic inclusions","volume":"20","author":"Huang","year":"2010","journal-title":"Brain Pathol."},{"key":"B90","doi-asserted-by":"publisher","first-page":"44834","DOI":"10.1074\/jbc.M408552200","article-title":"Domain architectures and characterization of an RNA-binding protein, TLS","volume":"279","author":"Iko","year":"2004","journal-title":"J. Biol. Chem."},{"key":"B91","doi-asserted-by":"publisher","first-page":"575","DOI":"10.1016\/j.neuron.2010.07.019","article-title":"Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS","volume":"67","author":"Israelson","year":"2010","journal-title":"Neuron"},{"key":"B92","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1002\/ana.22246","article-title":"Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS\/TLS","volume":"69","author":"Ito","year":"2011","journal-title":"Ann. Neurol."},{"key":"B93","doi-asserted-by":"publisher","first-page":"487","DOI":"10.1016\/j.cell.2015.12.038","article-title":"ATPase-modulated stress granules contain a diverse proteome and substructure","volume":"164","author":"Jain","year":"2016","journal-title":"Cell"},{"key":"B94","doi-asserted-by":"publisher","first-page":"542","DOI":"10.3109\/17482968.2010.482592","article-title":"A decrease in body mass index is associated with faster progression of motor symptoms and shorter survival in ALS","volume":"11","author":"Jawaid","year":"2010","journal-title":"Amyotroph. Lateral Scler."},{"key":"B95","doi-asserted-by":"publisher","first-page":"535","DOI":"10.1016\/j.neuron.2016.04.006","article-title":"Gain of Toxicity from ALS\/FTD-linked repeat expansions in C9ORF72 is alleviated by antisense oligonucleotides targeting GGGGCC-containing RNAs","volume":"90","author":"Jiang","year":"2016","journal-title":"Neuron"},{"key":"B96","doi-asserted-by":"publisher","first-page":"1226","DOI":"10.1038\/nn.4085","article-title":"Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD\/ALS","volume":"18","author":"Jovi\u010di\u0107","year":"2015","journal-title":"Nat. Neurosci."},{"key":"B97","doi-asserted-by":"publisher","first-page":"134","DOI":"10.1111\/jnc.13642","article-title":"Nuclear transport dysfunction: a common theme in amyotrophic lateral sclerosis and frontotemporal dementia","volume":"138","author":"Jovi\u010di\u0107","year":"2016","journal-title":"J. Neurochem."},{"key":"B99","doi-asserted-by":"publisher","first-page":"1668","DOI":"10.1016\/j.bbadis.2014.06.012","article-title":"AMPK-\u03b11 functions downstream of oxidative stress to mediate neuronal atrophy in Huntington\u2019s disease","volume":"1842","author":"Ju","year":"2014","journal-title":"Biochim. Biophys. Acta"},{"key":"B98","doi-asserted-by":"publisher","first-page":"209","DOI":"10.1083\/jcb.201105010","article-title":"Nuclear translocation of AMPK-\u03b11 potentiates striatal neurodegeneration in Huntington\u2019s disease","volume":"194","author":"Ju","year":"2011","journal-title":"J. Cell Biol."},{"key":"B100","doi-asserted-by":"publisher","first-page":"4107","DOI":"10.1007\/s00018-012-1025-2","article-title":"Energy dysfunction in Huntington\u2019s disease: insights from PGC-1\u03b1, AMPK and CKB","volume":"69","author":"Ju","year":"2012","journal-title":"Cell. Mol. Life Sci."},{"key":"B101","doi-asserted-by":"publisher","first-page":"2004","DOI":"10.1016\/j.bbadis.2016.07.015","article-title":"Proteomic analysis of FUS interacting proteins provides insights into FUS function and its role in ALS","volume":"1862","author":"Kamelgarn","year":"2016","journal-title":"Biochim. Biophys. Acta"},{"key":"B102","doi-asserted-by":"publisher","first-page":"513","DOI":"10.1016\/j.neuron.2004.07.022","article-title":"Kinesin transports RNA: isolation and characterization of an RNA-transporting granule","volume":"43","author":"Kanai","year":"2004","journal-title":"Neuron"},{"key":"B103","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1093\/ajcn\/63.1.130","article-title":"Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity of death","volume":"63","author":"Kasarskis","year":"1996","journal-title":"Am. J. Clin. Nutr."},{"key":"B104","doi-asserted-by":"publisher","first-page":"3347","DOI":"10.1073\/pnas.1112427109","article-title":"TDP-43 promotes microRNA biogenesis as a component of the Drosha and Dicer complexes","volume":"109","author":"Kawahara","year":"2012","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"B105","doi-asserted-by":"publisher","first-page":"790","DOI":"10.1093\/hmg\/ddw432","article-title":"Cytoplasmic poly-GA aggregates impair nuclear import of TDP-43 in C9orf72 ALS\/FTLD","volume":"26","author":"Khosravi","year":"2017","journal-title":"Hum. Mol. Genet."},{"key":"B106","doi-asserted-by":"publisher","first-page":"942","DOI":"10.1016\/S0140-6736(10)61156-7","article-title":"Amyotrophic lateral sclerosis","volume":"377","author":"Kiernan","year":"2011","journal-title":"Lancet"},{"key":"B107","doi-asserted-by":"publisher","first-page":"23246","DOI":"10.1074\/jbc.m114.577908","article-title":"Metformin suppresses lipopolysaccharide (LPS)-induced inflammatory response in murine macrophages via activating transcription factor-3 (ATF-3) induction","volume":"289","author":"Kim","year":"2014","journal-title":"J. Biol. Chem."},{"key":"B108","doi-asserted-by":"publisher","first-page":"65","DOI":"10.1016\/j.neuron.2013.02.029","article-title":"VCP is essential for mitochondrial quality control by PINK1\/Parkin and this function is impaired by VCP mutations","volume":"78","author":"Kim","year":"2013","journal-title":"Neuron"},{"key":"B109","doi-asserted-by":"publisher","first-page":"478","DOI":"10.1074\/jbc.M807017200","article-title":"Six classes of nuclear localization signals specific to different binding grooves of importin \u03b1","volume":"284","author":"Kosugi","year":"2009","journal-title":"J. Biol. Chem."},{"key":"B110","doi-asserted-by":"publisher","first-page":"366","DOI":"10.1038\/ncpneuro0853","article-title":"Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis","volume":"4","author":"K\u00fchnlein","year":"2008","journal-title":"Nat. Clin. Pract. Neurol."},{"key":"B111","doi-asserted-by":"publisher","first-page":"1205","DOI":"10.1126\/science.1166066","article-title":"Mutations in the FUS\/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis","volume":"323","author":"Kwiatkowski","year":"2009","journal-title":"Science"},{"key":"B113","doi-asserted-by":"publisher","first-page":"1112","DOI":"10.1212\/wnl.58.7.1112","article-title":"APOE: a potential marker of disease progression in ALS","volume":"58","author":"Lacomblez","year":"2002","journal-title":"Neurology"},{"key":"B114","doi-asserted-by":"publisher","first-page":"E4530","DOI":"10.1073\/pnas.1318835110","article-title":"Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration","volume":"110","author":"Lagier-Tourenne","year":"2013","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"B115","doi-asserted-by":"publisher","first-page":"R46","DOI":"10.1093\/hmg\/ddq137","article-title":"TDP-43 and FUS\/TLS: emerging roles in RNA processing and neurodegeneration","volume":"19","author":"Lagier-Tourenne","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"B116","doi-asserted-by":"publisher","first-page":"1488","DOI":"10.1038\/nn.3230","article-title":"Divergent roles of ALS-linked proteins FUS\/TLS and TDP-43 intersect in processing long pre-mRNAs","volume":"15","author":"Lagier-Tourenne","year":"2012","journal-title":"Nat. Neurosci."},{"key":"B112","doi-asserted-by":"publisher","first-page":"77","DOI":"10.1038\/352077a0","article-title":"Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy","volume":"352","author":"La Spada","year":"1991","journal-title":"Nature"},{"key":"B118","doi-asserted-by":"publisher","first-page":"1178","DOI":"10.1016\/j.celrep.2013.10.049","article-title":"Hexanucleotide repeats in ALS\/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxic","volume":"5","author":"Lee","year":"2013","journal-title":"Cell Rep."},{"key":"B117","doi-asserted-by":"publisher","first-page":"774.e17","DOI":"10.1016\/j.cell.2016.10.002","article-title":"C9orf72 dipeptide repeats impair the assembly, dynamics, and function of membrane-less organelles","volume":"167","author":"Lee","year":"2016","journal-title":"Cell"},{"key":"B119","doi-asserted-by":"publisher","first-page":"755","DOI":"10.1242\/dmm.020099","article-title":"ALS mutant FUS proteins are recruited into stress granules in induced pluripotent stem cell-derived motoneurons","volume":"8","author":"Lenzi","year":"2015","journal-title":"Dis. Model. Mech."},{"key":"B120","doi-asserted-by":"publisher","first-page":"499","DOI":"10.1093\/bioinformatics\/bts725","article-title":"The product of C9orf72, a gene strongly implicated in neurodegeneration, is structurally related to DENN Rab-GEFs","volume":"29","author":"Levine","year":"2013","journal-title":"Bioinformatics"},{"key":"B121","doi-asserted-by":"publisher","first-page":"82","DOI":"10.1016\/j.bcmd.2010.03.002","article-title":"TEThered to Runx: novel binding partners for runx factors","volume":"45","author":"Li","year":"2010","journal-title":"Blood Cells Mol. Dis."},{"key":"B122","doi-asserted-by":"publisher","first-page":"361","DOI":"10.1083\/jcb.201302044","article-title":"Stress granules as crucibles of ALS pathogenesis","volume":"201","author":"Li","year":"2013","journal-title":"J. Cell Biol."},{"key":"B123","doi-asserted-by":"publisher","first-page":"1123","DOI":"10.1523\/JNEUROSCI.6554-10.2012","article-title":"Reduced activity of AMP-activated protein kinase protects against genetic models of motor neuron disease","volume":"32","author":"Lim","year":"2012","journal-title":"J. Neurosci."},{"key":"B124","doi-asserted-by":"publisher","first-page":"789.e12","DOI":"10.1016\/j.cell.2016.10.003","article-title":"Toxic PR poly-dipeptides encoded by the C9orf72 repeat expansion target LC domain polymers","volume":"167","author":"Lin","year":"2016","journal-title":"Cell"},{"key":"B125","doi-asserted-by":"publisher","first-page":"416","DOI":"10.1016\/j.neuron.2013.07.033","article-title":"Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis","volume":"79","author":"Ling","year":"2013","journal-title":"Neuron"},{"key":"B128","doi-asserted-by":"publisher","first-page":"787","DOI":"10.1093\/hmg\/ddu497","article-title":"Activation of AMP-activated protein kinase \u03b11 mediates mislocalization of TDP-43 in amyotrophic lateral sclerosis","volume":"24","author":"Liu","year":"","journal-title":"Hum. Mol. Genet."},{"key":"B127","doi-asserted-by":"publisher","first-page":"432","DOI":"10.1016\/j.febslet.2014.12.029","article-title":"Aberrant activation of AMP-activated protein kinase contributes to the abnormal distribution of HuR in amyotrophic lateral sclerosis","volume":"589","author":"Liu","year":"","journal-title":"FEBS Lett."},{"key":"B126","doi-asserted-by":"publisher","first-page":"222","DOI":"10.2174\/15672026113109990060","article-title":"Mitochondrial fusion and fission proteins expression dynamically change in a murine model of amyotrophic lateral sclerosis","volume":"10","author":"Liu","year":"2013","journal-title":"Curr. Neurovasc. Res."},{"key":"B129","doi-asserted-by":"publisher","first-page":"e13250","DOI":"10.1371\/journal.pone.0013250","article-title":"Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue","volume":"5","author":"Liu-Yesucevitz","year":"2010","journal-title":"PLoS One"},{"key":"B130","doi-asserted-by":"publisher","first-page":"181","DOI":"10.3389\/fncel.2013.00181","article-title":"Cellular and molecular mechanisms involved in the neuroprotective effects of VEGF on motoneurons","volume":"7","author":"Llad\u00f3","year":"2013","journal-title":"Front. Cell. Neurosci."},{"key":"B131","doi-asserted-by":"publisher","first-page":"1776","DOI":"10.1172\/JCI29044","article-title":"AMP-activated protein kinase signaling in metabolic regulation","volume":"116","author":"Long","year":"2006","journal-title":"J. Clin. Invest."},{"key":"B132","doi-asserted-by":"publisher","first-page":"383","DOI":"10.1016\/j.neuron.2016.09.015","article-title":"Poly(GR) in C9ORF72-related ALS\/FTD compromises mitochondrial function and increases oxidative stress and DNA damage in iPSC-derived motor neurons","volume":"92","author":"Lopez-Gonzalez","year":"2016","journal-title":"Neuron"},{"key":"B135","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1186\/1756-6606-2-30","article-title":"Frontotemporal dementia and amyotrophic lateral sclerosis-associated disease protein TDP-43 promotes dendritic branching","volume":"2","author":"Lu","year":"2009","journal-title":"Mol. Brain"},{"key":"B134","doi-asserted-by":"publisher","first-page":"33989","DOI":"10.1074\/jbc.M109.067918","article-title":"Amyotrophic lateral sclerosis-linked mutant SOD1 sequesters Hu antigen R (HuR) and TIA-1-related protein (TIAR): implications for impaired post-transcriptional regulation of vascular endothelial growth factor","volume":"284","author":"Lu","year":"2009","journal-title":"J. Biol. Chem."},{"key":"B133","doi-asserted-by":"publisher","first-page":"31792","DOI":"10.1074\/jbc.M114.573246","article-title":"Hu antigen R (HuR) is a positive regulator of the RNA-binding proteins TDP-43 and FUS\/TLS: implications for amyotrophic lateral sclerosis","volume":"289","author":"Lu","year":"2014","journal-title":"J. Biol. Chem."},{"key":"B136","doi-asserted-by":"publisher","first-page":"12230","DOI":"10.1523\/JNEUROSCI.1694-14.2014","article-title":"Inhibition of AMP-activated protein kinase signaling alleviates impairments in hippocampal synaptic plasticity induced by amyloid \u03b2","volume":"34","author":"Ma","year":"2014","journal-title":"J. Neurosci."},{"key":"B137","doi-asserted-by":"publisher","first-page":"1725","DOI":"10.1016\/j.bbamcr.2015.03.015","article-title":"5\u2032-AMP-activated protein kinase \u03b1 regulates stress granule biogenesis","volume":"1853","author":"Mahboubi","year":"","journal-title":"Biochim. Biophys. Acta"},{"key":"B138","doi-asserted-by":"publisher","first-page":"54","DOI":"10.1016\/j.dib.2015.04.010","article-title":"Data in support of 5\u2032AMP-activated protein kinase \u03b1 regulates stress granule biogenesis","volume":"4","author":"Mahboubi","year":"","journal-title":"Data Brief"},{"key":"B139","doi-asserted-by":"publisher","first-page":"94","DOI":"10.1016\/j.neuron.2013.02.003","article-title":"The CAMKK2-AMPK kinase pathway mediates the synaptotoxic effects of A\u03b2 oligomers through Tau phosphorylation","volume":"78","author":"Mairet-Coello","year":"2013","journal-title":"Neuron"},{"key":"B140","doi-asserted-by":"publisher","first-page":"7131","DOI":"10.1523\/jneurosci.1801-06.2006","article-title":"RNA trafficking and local protein synthesis in dendrites: an overview","volume":"26","author":"Martin","year":"2006","journal-title":"J. Neurosci."},{"key":"B141","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1385\/NMM:9:1:17","article-title":"Energy intake and amyotrophic lateral sclerosis","volume":"9","author":"Mattson","year":"2007","journal-title":"Neuromolecular Med."},{"key":"B142","doi-asserted-by":"publisher","first-page":"1400","DOI":"10.1093\/hmg\/ddr021","article-title":"TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1","volume":"20","author":"McDonald","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"B143","doi-asserted-by":"publisher","first-page":"837","DOI":"10.1097\/NEN.0000000000000102","article-title":"Poly-A binding protein-1 localization to a subset of TDP-43 inclusions in amyotrophic lateral sclerosis occurs more frequently in patients harboring an expansion in C9orf72","volume":"73","author":"McGurk","year":"2014","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"B144","doi-asserted-by":"publisher","first-page":"258","DOI":"10.1136\/jmedgenet-2011-100699","article-title":"Phenotype difference between ALS patients with expanded repeats in C9ORF72 and patients with mutations in other ALS-related genes","volume":"49","author":"Millecamps","year":"2012","journal-title":"J. Med. Genet."},{"key":"B145","doi-asserted-by":"publisher","first-page":"4439","DOI":"10.1093\/emboj\/19.17.4439","article-title":"Recruitment of human muscleblind proteins to (CUG)n expansions associated with myotonic dystrophy","volume":"19","author":"Miller","year":"2000","journal-title":"EMBO J."},{"key":"B146","doi-asserted-by":"publisher","first-page":"69","DOI":"10.1093\/jb\/mvw036","article-title":"Importin \u03b1: a key molecule in nuclear transport and non-transport functions","volume":"160","author":"Miyamoto","year":"2016","journal-title":"J. Biochem."},{"key":"B147","doi-asserted-by":"publisher","first-page":"413","DOI":"10.1007\/s00401-013-1088-7","article-title":"hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive\/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations","volume":"125","author":"Mori","year":"","journal-title":"Acta Neuropathol."},{"key":"B148","doi-asserted-by":"publisher","first-page":"1335","DOI":"10.1126\/science.1232927","article-title":"The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD\/ALS","volume":"339","author":"Mori","year":"","journal-title":"Science"},{"key":"B149","doi-asserted-by":"publisher","first-page":"3531","DOI":"10.1128\/MCB.05124-11","article-title":"Hypoxia triggers AMPK activation through reactive oxygen species-mediated activation of calcium release-activated calcium channels","volume":"31","author":"Mungai","year":"2011","journal-title":"Mol. Cell. Biol."},{"key":"B150","first-page":"2154","article-title":"HuR, a RNA stability factor, is expressed in malignant brain tumors and binds to adenine- and uridine-rich elements within the 3\u2032 untranslated regions of cytokine and angiogenic factor mRNAs","volume":"61","author":"Nabors","year":"2001","journal-title":"Cancer Res."},{"key":"B151","doi-asserted-by":"publisher","first-page":"456","DOI":"10.4161\/rna.23547","article-title":"Paraspeckle formation during the biogenesis of long non-coding RNAs","volume":"10","author":"Naganuma","year":"2013","journal-title":"RNA Biol."},{"key":"B152","doi-asserted-by":"publisher","first-page":"130","DOI":"10.1126\/science.1134108","article-title":"Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis","volume":"314","author":"Neumann","year":"2006","journal-title":"Science"},{"key":"B153","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1186\/s13024-016-0136-x","article-title":"Chronic adiponectin deficiency leads to Alzheimer\u2019s disease-like cognitive impairments and pathologies through AMPK inactivation and cerebral insulin resistance in aged mice","volume":"11","author":"Ng","year":"2016","journal-title":"Mol. Neurodegener."},{"key":"B154","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1186\/1756-6606-6-31","article-title":"The long non-coding RNA nuclear-enriched abundant transcript 1_2 induces paraspeckle formation in the motor neuron during the early phase of amyotrophic lateral sclerosis","volume":"6","author":"Nishimoto","year":"2013","journal-title":"Mol. Brain"},{"key":"B155","doi-asserted-by":"publisher","first-page":"1097","DOI":"10.1126\/science.252.5009.1097","article-title":"Instability of a 550-base pair DNA segment and abnormal methylation in fragile X syndrome","volume":"252","author":"Oberl\u00e9","year":"1991","journal-title":"Science"},{"key":"B156","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1186\/s40478-016-0316-5","article-title":"Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts","volume":"4","author":"Onesto","year":"2016","journal-title":"Acta Neuropathol. Commun."},{"key":"B157","doi-asserted-by":"publisher","first-page":"131","DOI":"10.1038\/88842","article-title":"Deletion of the hypoxia-response element in the vascular endothelial growth factor promoter causes motor neuron degeneration","volume":"28","author":"Oosthuyse","year":"2001","journal-title":"Nat. Genet."},{"key":"B158","doi-asserted-by":"publisher","first-page":"415","DOI":"10.1016\/j.neuint.2012.01.019","article-title":"Endogenous TDP-43 localized to stress granules can subsequently form protein aggregates","volume":"60","author":"Parker","year":"2012","journal-title":"Neurochem. Int."},{"key":"B159","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1016\/j.brainres.2012.02.059","article-title":"Misregulated RNA processing in amyotrophic lateral sclerosis","volume":"1462","author":"Polymenidou","year":"2012","journal-title":"Brain Res."},{"key":"B160","doi-asserted-by":"publisher","first-page":"459","DOI":"10.1038\/nn.2779","article-title":"Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43","volume":"14","author":"Polymenidou","year":"2011","journal-title":"Nat. Neurosci."},{"key":"B161","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1210\/mend.12.1.0043","article-title":"TLS (translocated-in-liposarcoma) is a high-affinity interactor for steroid, thyroid hormone and retinoid receptors","volume":"12","author":"Powers","year":"1998","journal-title":"Mol. Endocrinol."},{"key":"B162","doi-asserted-by":"publisher","first-page":"668","DOI":"10.1016\/j.tcb.2016.05.004","article-title":"Principles and properties of stress granules","volume":"26","author":"Protter","year":"2016","journal-title":"Trends Cell Biol."},{"key":"B163","doi-asserted-by":"publisher","first-page":"727","DOI":"10.1016\/j.cell.2013.07.038","article-title":"Altered ribostasis: RNA-protein granules in degenerative disorders","volume":"154","author":"Ramaswami","year":"2013","journal-title":"Cell"},{"key":"B164","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1016\/j.neuron.2011.09.010","article-title":"A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD","volume":"72","author":"Renton","year":"2011","journal-title":"Neuron"},{"key":"B166","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1111\/j.1469-7793.2000.00003.x","article-title":"Mitochondrial uncoupling proteins: from mitochondria to the regulation of energy balance","volume":"529","author":"Ricquier","year":"2000","journal-title":"J. Physiol."},{"key":"B167","doi-asserted-by":"publisher","first-page":"128","DOI":"10.1016\/j.neulet.2007.03.066","article-title":"Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS","volume":"420","author":"Robertson","year":"2007","journal-title":"Neurosci. Lett."},{"key":"B168","doi-asserted-by":"publisher","first-page":"603","DOI":"10.1038\/srep00603","article-title":"Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain","volume":"2","author":"Rogelj","year":"2012","journal-title":"Sci. Rep."},{"key":"B169","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1016\/S1474-4422(14)70233-9","article-title":"C9orf72 expansions in frontotemporal dementia and amyotrophic lateral sclerosis","volume":"14","author":"Rohrer","year":"2015","journal-title":"Lancet Neurol."},{"key":"B170","doi-asserted-by":"publisher","first-page":"1787","DOI":"10.1242\/jcs.165332","article-title":"Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS","volume":"128","author":"Rossi","year":"2015","journal-title":"J. Cell Sci."},{"key":"B171","doi-asserted-by":"publisher","first-page":"2222","DOI":"10.1002\/jcp.24395","article-title":"FUS\/TLS assembles into stress granules and is a prosurvival factor during hyperosmolar stress","volume":"228","author":"Sama","year":"2013","journal-title":"J. Cell. Physiol."},{"key":"B173","doi-asserted-by":"publisher","first-page":"1204","DOI":"10.1074\/jbc.M110.190884","article-title":"Identification of neuronal RNA targets of TDP-43-containing ribonucleoprotein complexes","volume":"286","author":"Sephton","year":"2011","journal-title":"J. Biol. Chem."},{"key":"B172","doi-asserted-by":"publisher","first-page":"3621","DOI":"10.1007\/s00018-015-1943-x","article-title":"The function of RNA-binding proteins at the synapse: implications for neurodegeneration","volume":"72","author":"Sephton","year":"2015","journal-title":"Cell. Mol. Life Sci."},{"key":"B175","doi-asserted-by":"publisher","first-page":"16325","DOI":"10.1073\/pnas.1003459107","article-title":"Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice","volume":"107","author":"Shan","year":"2010","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"B174","doi-asserted-by":"publisher","first-page":"70","DOI":"10.1016\/j.neulet.2009.04.031","article-title":"Mislocalization of TDP-43 in the G93A mutant SOD1 transgenic mouse model of ALS","volume":"458","author":"Shan","year":"2009","journal-title":"Neurosci. Lett."},{"key":"B176","doi-asserted-by":"publisher","first-page":"2298","DOI":"10.1093\/hmg\/ddt622","article-title":"Compromised paraspeckle formation as a pathogenic factor in FUSopathies","volume":"23","author":"Shelkovnikova","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"B177","doi-asserted-by":"publisher","first-page":"1390","DOI":"10.1016\/j.bbadis.2013.03.002","article-title":"RNA toxicity in human disease and animal models: from the uncovering of a new mechanism to the development of promising therapies","volume":"1832","author":"Sicot","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"B178","doi-asserted-by":"publisher","first-page":"723","DOI":"10.1093\/brain\/awr353","article-title":"The clinical and pathological phenotype of C9ORF72 hexanucleotide repeat expansions","volume":"135","author":"Sim\u00f3n-S\u00e1nchez","year":"2012","journal-title":"Brain"},{"key":"B179","doi-asserted-by":"publisher","first-page":"520","DOI":"10.1111\/j.1365-2796.2009.02173.x","article-title":"Statins and amyotrophic lateral sclerosis\u2013the level of evidence for an association","volume":"266","author":"S\u00f8rensen","year":"2009","journal-title":"J. Intern. Med."},{"key":"B180","doi-asserted-by":"publisher","first-page":"309","DOI":"10.1002\/ana.24012","article-title":"Amyotrophic lateral sclerosis: problems and prospects","volume":"74","author":"Sreedharan","year":"2013","journal-title":"Ann. Neurol."},{"key":"B181","doi-asserted-by":"publisher","first-page":"66","DOI":"10.1212\/wnl.50.1.66","article-title":"Prognostic indicators of survival in ALS. ALS CNTF Treatment Study Group","volume":"50","author":"Stambler","year":"1998","journal-title":"Neurology"},{"key":"B182","doi-asserted-by":"publisher","first-page":"9124","DOI":"10.1038\/srep09124","article-title":"Exome sequencing of case-unaffected-parents trios reveals recessive and de novo genetic variants in sporadic ALS","volume":"5","author":"Steinberg","year":"2015","journal-title":"Sci. Rep."},{"key":"B183","doi-asserted-by":"publisher","first-page":"320","DOI":"10.1016\/j.mcn.2007.03.007","article-title":"TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein","volume":"35","author":"Strong","year":"2007","journal-title":"Mol. Cell. Neurosci."},{"key":"B184","doi-asserted-by":"publisher","first-page":"815","DOI":"10.1128\/MCB.00763-12","article-title":"Stress granules inhibit apoptosis by reducing reactive oxygen species production","volume":"33","author":"Takahashi","year":"2013","journal-title":"Mol. Cell. Biol."},{"key":"B185","doi-asserted-by":"publisher","first-page":"1457","DOI":"10.1111\/j.1471-4159.2009.06225.x","article-title":"A protein-protein interaction of stress-responsive myosin VI endowed to inhibit neural progenitor self-replication with RNA binding protein, TLS, in murine hippocampus","volume":"110","author":"Takarada","year":"2009","journal-title":"J. Neurochem."},{"key":"B186","doi-asserted-by":"publisher","first-page":"197","DOI":"10.1038\/nature20413","article-title":"Decoding ALS: from genes to mechanism","volume":"539","author":"Taylor","year":"2016","journal-title":"Nature"},{"key":"B187","doi-asserted-by":"publisher","first-page":"1064","DOI":"10.1097\/NEN.0b013e318275df4b","article-title":"Decreased mRNA expression of PGC-1\u03b1 and PGC-1\u03b1-regulated factors in the SOD1G93A ALS mouse model and in human sporadic ALS","volume":"71","author":"Thau","year":"2012","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"B188","doi-asserted-by":"publisher","first-page":"503","DOI":"10.1042\/BJ20101485","article-title":"AMP-activated protein kinase (AMPK) is a tau kinase, activated in response to amyloid \u03b2-peptide exposure","volume":"434","author":"Thornton","year":"2011","journal-title":"Biochem. J."},{"key":"B189","doi-asserted-by":"publisher","first-page":"452","DOI":"10.1038\/nn.2778","article-title":"Characterizing the RNA targets and position-dependent splicing regulation by TDP-43","volume":"14","author":"Tollervey","year":"2011","journal-title":"Nat. Neurosci."},{"key":"B190","doi-asserted-by":"publisher","first-page":"e0145425","DOI":"10.1371\/journal.pone.0150682","article-title":"SIRT1 activity is linked to its brain region-specific phosphorylation and is impaired in Huntington\u2019s disease mice","volume":"11","author":"Tulino","year":"2016","journal-title":"PLoS One"},{"key":"B191","doi-asserted-by":"publisher","first-page":"13395","DOI":"10.1074\/jbc.M011176200","article-title":"Involvement of the pro-oncoprotein TLS (translocated in liposarcoma) in nuclear factor-\u03ba B p65-mediated transcription as a coactivator","volume":"276","author":"Uranishi","year":"2001","journal-title":"J. Biol. Chem."},{"key":"B192","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1016\/j.jns.2008.12.027","article-title":"Do patients with amyotrophic lateral sclerosis (ALS) have increased energy needs?","volume":"279","author":"Vaisman","year":"2009","journal-title":"J. Neurol. Sci."},{"key":"B193","doi-asserted-by":"publisher","first-page":"430","DOI":"10.3109\/17482961003605796","article-title":"Job strain, hypoxia and risk of amyotrophic lateral sclerosis: results from a death certificate study","volume":"11","author":"Vanacore","year":"2010","journal-title":"Amyotroph. Lateral Scler."},{"key":"B194","doi-asserted-by":"publisher","first-page":"1208","DOI":"10.1126\/science.1165942","article-title":"Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6","volume":"323","author":"Vance","year":"2009","journal-title":"Science"},{"key":"B195","doi-asserted-by":"publisher","first-page":"1043","DOI":"10.1093\/hmg\/ddv513","article-title":"AMPK activation protects from neuronal dysfunction and vulnerability across nematode, cellular and mouse models of Huntington\u2019s disease","volume":"25","author":"V\u00e1zquez-Manrique","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"B196","doi-asserted-by":"publisher","first-page":"168","DOI":"10.1016\/j.brainres.2009.09.105","article-title":"Tar DNA binding protein of 43 kDa (TDP-43), 14\u20133-3 proteins and copper\/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS)","volume":"1305","author":"Volkening","year":"2009","journal-title":"Brain Res."},{"key":"B200","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.brainres.2016.06.043","article-title":"scAAV9-VEGF prolongs the survival of transgenic ALS mice by promoting activation of M2 microglia and the PI3K\/Akt pathway","volume":"1648","author":"Wang","year":"2016","journal-title":"Brain Res."},{"key":"B198","doi-asserted-by":"publisher","first-page":"4706","DOI":"10.1093\/hmg\/ddt319","article-title":"The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons","volume":"22","author":"Wang","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"B197","doi-asserted-by":"publisher","first-page":"797","DOI":"10.1111\/j.1471-4159.2007.05190.x","article-title":"TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor","volume":"105","author":"Wang","year":"2008","journal-title":"J. Neurochem."},{"key":"B199","doi-asserted-by":"publisher","first-page":"48376","DOI":"10.1074\/jbc.m409014200","article-title":"AMP-activated protein kinase-regulated phosphorylation and acetylation of importin \u03b11: involvement in the nuclear import of RNA-binding protein HuR","volume":"279","author":"Wang","year":"2004","journal-title":"J. Biol. Chem."},{"key":"B201","doi-asserted-by":"publisher","first-page":"1421","DOI":"10.15252\/emmm.201606403","article-title":"Mitochondria-associated membrane collapse is a common pathomechanism in SIGMAR1- and SOD1-linked ALS","volume":"8","author":"Watanabe","year":"2016","journal-title":"EMBO Mol. Med."},{"key":"B202","doi-asserted-by":"publisher","first-page":"769","DOI":"10.1016\/j.molmed.2016.07.005","article-title":"Common molecular pathways in amyotrophic lateral sclerosis and Frontotemporal dementia","volume":"22","author":"Weishaupt","year":"2016","journal-title":"Trends Mol. Med."},{"key":"B203","doi-asserted-by":"publisher","first-page":"2944","DOI":"10.1101\/gad.848200","article-title":"Cap methyltransferase selective binding and methylation of GpppG-RNA are stimulated by importin-\u03b1","volume":"14","author":"Wen","year":"2000","journal-title":"Genes Dev."},{"key":"B204","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1126\/science.aad2033","article-title":"Cytoplasmic protein aggregates interfere with nucleocytoplasmic transport of protein and RNA","volume":"351","author":"Woerner","year":"2016","journal-title":"Science"},{"key":"B205","doi-asserted-by":"publisher","first-page":"83","DOI":"10.1093\/hmg\/ddv450","article-title":"Folliculin, a tumor suppressor associated with Birt-Hogg-Dube (BHD) syndrome, is a novel modifier of TDP-43 cytoplasmic translocation and aggregation","volume":"25","author":"Xia","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"B206","doi-asserted-by":"publisher","first-page":"10851","DOI":"10.1523\/JNEUROSCI.1630-10.2010","article-title":"Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits and early mortality in transgenic mice","volume":"30","author":"Xu","year":"2010","journal-title":"J. Neurosci."},{"key":"B207","doi-asserted-by":"publisher","first-page":"1039","DOI":"10.1007\/s00415-011-6292-6","article-title":"Sporadic juvenile amyotrophic lateral sclerosis caused by mutant FUS\/TLS: possible association of mental retardation with this mutation","volume":"259","author":"Yamashita","year":"2012","journal-title":"J. Neurol."},{"key":"B208","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1016\/j.expneurol.2009.08.011","article-title":"Dynamics of axonal mRNA transport and implications for peripheral nerve regeneration","volume":"223","author":"Yoo","year":"2010","journal-title":"Exp. Neurol."},{"key":"B209","doi-asserted-by":"publisher","first-page":"2345","DOI":"10.1016\/j.cub.2006.10.024","article-title":"Myosin-Va facilitates the accumulation of mRNA\/protein complex in dendritic spines","volume":"16","author":"Yoshimura","year":"2006","journal-title":"Curr. Biol."},{"key":"B211","doi-asserted-by":"publisher","first-page":"56","DOI":"10.1038\/nature14973","article-title":"The C9orf72 repeat expansion disrupts nucleocytoplasmic transport","volume":"525","author":"Zhang","year":"2015","journal-title":"Nature"},{"key":"B212","doi-asserted-by":"publisher","first-page":"668","DOI":"10.1038\/nn.4272","article-title":"C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins","volume":"19","author":"Zhang","year":"2016","journal-title":"Nat. Neurosci."},{"key":"B210","doi-asserted-by":"publisher","first-page":"283","DOI":"10.3389\/fgene.2012.00283","article-title":"Discovery of novel DENN proteins: implications for the evolution of Eukaryotic intracellular membrane structures and human disease","volume":"3","author":"Zhang","year":"2012","journal-title":"Front. Genet."}],"container-title":["Frontiers in Cellular Neuroscience"],"original-title":[],"link":[{"URL":"http:\/\/journal.frontiersin.org\/article\/10.3389\/fncel.2017.00126\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,9,23]],"date-time":"2019-09-23T02:09:13Z","timestamp":1569204553000},"score":1,"resource":{"primary":{"URL":"http:\/\/journal.frontiersin.org\/article\/10.3389\/fncel.2017.00126\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,5,4]]},"references-count":211,"alternative-id":["10.3389\/fncel.2017.00126"],"URL":"https:\/\/doi.org\/10.3389\/fncel.2017.00126","relation":{},"ISSN":["1662-5102"],"issn-type":[{"value":"1662-5102","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,5,4]]},"article-number":"126"}}