{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T08:53:51Z","timestamp":1787734431969,"version":"build-2784847793"},"update-to":[{"DOI":"10.1371\/journal.pgen.1006680","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2017,4,14]],"date-time":"2017-04-14T00:00:00Z","timestamp":1492128000000}}],"reference-count":49,"publisher":"Public Library of Science (PLoS)","issue":"3","license":[{"start":{"date-parts":[[2017,3,31]],"date-time":"2017-03-31T00:00:00Z","timestamp":1490918400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100010790","name":"FightSMA","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010790","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005202","name":"Muscular Dystrophy Association","doi-asserted-by":"publisher","award":["MDA294433"],"award-info":[{"award-number":["MDA294433"]}],"id":[{"id":"10.13039\/100005202","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000723","name":"Tenovus","doi-asserted-by":"publisher","award":["E15\/4"],"award-info":[{"award-number":["E15\/4"]}],"id":[{"id":"10.13039\/501100000723","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000871","name":"Newlife Foundation for Disabled Children","doi-asserted-by":"publisher","award":["SG\/14-15\/08"],"award-info":[{"award-number":["SG\/14-15\/08"]}],"id":[{"id":"10.13039\/501100000871","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Gwendolyn Strong Foundation"},{"DOI":"10.13039\/100004440","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["Pennetta8260"],"award-info":[{"award-number":["Pennetta8260"]}],"id":[{"id":"10.13039\/100004440","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000406","name":"Motor Neurone Disease Association","doi-asserted-by":"publisher","award":["Pennetta6231"],"award-info":[{"award-number":["Pennetta6231"]}],"id":[{"id":"10.13039\/501100000406","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005202","name":"Muscular Dystrophy Association","doi-asserted-by":"publisher","award":["382231"],"award-info":[{"award-number":["382231"]}],"id":[{"id":"10.13039\/100005202","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010790","name":"FightSMA","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010790","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Gwendolyn Strong Foundation"},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["T32 GM008396"],"award-info":[{"award-number":["T32 GM008396"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["www.plosgenetics.org"],"crossmark-restriction":false},"short-container-title":["PLoS Genet"],"DOI":"10.1371\/journal.pgen.1006680","type":"journal-article","created":{"date-parts":[[2017,3,31]],"date-time":"2017-03-31T14:25:32Z","timestamp":1490970332000},"page":"e1006680","update-policy":"https:\/\/doi.org\/10.1371\/journal.pgen.corrections_policy","source":"Crossref","is-referenced-by-count":41,"title":["Comparison of independent screens on differentially vulnerable motor neurons reveals alpha-synuclein as a common modifier in motor neuron diseases"],"prefix":"10.1371","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1692-2208","authenticated-orcid":true,"given":"Rachel A.","family":"Kline","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin A.","family":"Kaifer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erkan Y.","family":"Osman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Carella","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ariana","family":"Tiberi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jolill","family":"Ross","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppa","family":"Pennetta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian L.","family":"Lorson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6841-702X","authenticated-orcid":true,"given":"Lyndsay M.","family":"Murray","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2017,3,31]]},"reference":[{"issue":"11","key":"ref1","doi-asserted-by":"publisher","first-page":"769","DOI":"10.1038\/nrg2680","article-title":"Genetics of motor neuron disorders: new insights into pathogenic mechanisms","volume":"10","author":"PA Dion","year":"2009","journal-title":"Nat Rev Genet"},{"issue":"1","key":"ref2","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1016\/0092-8674(95)90460-3","article-title":"Identification and characterization of a spinal muscular atrophy-determining gene","volume":"80","author":"S Lefebvre","year":"1995","journal-title":"Cell"},{"issue":"4","key":"ref3","doi-asserted-by":"publisher","first-page":"631","DOI":"10.1093\/hmg\/4.4.631","article-title":"Deletions in the survival motor neuron gene on 5q13 in autosomal recessive spinal muscular atrophy","volume":"4","author":"NR Rodrigues","year":"1995","journal-title":"Hum Mol Genet"},{"issue":"3","key":"ref4","doi-asserted-by":"publisher","first-page":"230","DOI":"10.1038\/3030","article-title":"Differential SMN2 expression associated with SMA severity","volume":"20","author":"DK Gavrilov","year":"1998","journal-title":"Nat Genet"},{"key":"ref5","article-title":"GeneReviews(R)","author":"A La Spada","year":"1993"},{"issue":"7","key":"ref6","doi-asserted-by":"publisher","first-page":"1424","DOI":"10.1002\/cne.23917","article-title":"Cross-disease comparison of amyotrophic lateral sclerosis and spinal muscular atrophy reveals conservation of selective vulnerability but differential neuromuscular junction pathology","volume":"524","author":"LH Comley","year":"2016","journal-title":"J Comp Neurol"},{"issue":"1","key":"ref7","doi-asserted-by":"publisher","first-page":"185","DOI":"10.1093\/hmg\/ddr453","article-title":"Severe neuromuscular denervation of clinically relevant muscles in a mouse model of spinal muscular atrophy","volume":"21","author":"KK Ling","year":"2012","journal-title":"Hum Mol Genet"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1016\/j.nbd.2012.08.019","article-title":"Defects in neuromuscular junction remodelling in the Smn(2B\/-) mouse model of spinal muscular atrophy","volume":"49","author":"LM Murray","year":"2013","journal-title":"Neurobiol Dis"},{"issue":"7","key":"ref9","doi-asserted-by":"publisher","first-page":"949","DOI":"10.1093\/hmg\/ddm367","article-title":"Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophy","volume":"17","author":"LM Murray","year":"2008","journal-title":"Hum Mol Genet"},{"issue":"9","key":"ref10","doi-asserted-by":"publisher","first-page":"644","DOI":"10.1212\/01.wnl.0000324623.89105.c4","article-title":"Natural history of SMA IIIb: muscle strength decreases in a predictable sequence and magnitude","volume":"71","author":"F Deymeer","year":"2008","journal-title":"Neurology"},{"issue":"1","key":"ref11","doi-asserted-by":"publisher","first-page":"61","DOI":"10.1017\/S0012162200000116","article-title":"New ocular movement detector system as a communication tool in ventilator-assisted Werdnig-Hoffmann disease","volume":"42","author":"M Kubota","year":"2000","journal-title":"Dev Med Child Neurol"},{"issue":"5","key":"ref12","doi-asserted-by":"publisher","first-page":"1037","DOI":"10.1212\/WNL.42.5.1037","article-title":"Ocular motor function in motor neuron disease","volume":"42","author":"M Gizzi","year":"1992","journal-title":"Neurology"},{"issue":"1","key":"ref13","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1016\/S0304-3940(02)01140-0","article-title":"Differential vulnerability of cranial motoneurons in mouse models with motor neuron degeneration","volume":"335","author":"C Haenggeli","year":"2002","journal-title":"Neurosci Lett"},{"issue":"1","key":"ref14","doi-asserted-by":"publisher","first-page":"112","DOI":"10.1002\/(SICI)1096-9861(20000103)416:1<112::AID-CNE9>3.0.CO;2-K","article-title":"Differential vulnerability of oculomotor, facial, and hypoglossal nuclei in G86R superoxide dismutase transgenic mice","volume":"416","author":"EA Nimchinsky","year":"2000","journal-title":"J Comp Neurol"},{"issue":"6","key":"ref15","doi-asserted-by":"publisher","first-page":"1593","DOI":"10.1042\/BST20130142","article-title":"The intriguing case of motor neuron disease: ALS and SMA come closer","volume":"41","author":"T Achsel","year":"2013","journal-title":"Biochem Soc Trans"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1186\/s40478-015-0231-1","article-title":"Transcriptional profiling of differentially vulnerable motor neurons at pre-symptomatic stage in the Smn (2b\/-) mouse model of spinal muscular atrophy","volume":"3","author":"LM Murray","year":"2015","journal-title":"Acta Neuropathol Commun"},{"issue":"1","key":"ref17","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1007\/s00401-012-1058-5","article-title":"Unravelling the enigma of selective vulnerability in neurodegeneration: motor neurons resistant to degeneration in ALS show distinct gene expression characteristics and decreased susceptibility to excitotoxicity","volume":"125","author":"A Brockington","year":"2013","journal-title":"Acta Neuropathol"},{"issue":"Pt 8","key":"ref18","doi-asserted-by":"publisher","first-page":"2313","DOI":"10.1093\/brain\/awq167","article-title":"Global gene expression profiling of somatic motor neuron populations with different vulnerability identify molecules and pathways of degeneration and protection","volume":"133","author":"E Hedlund","year":"2010","journal-title":"Brain"},{"issue":"2","key":"ref19","doi-asserted-by":"publisher","first-page":"333","DOI":"10.1016\/j.neuron.2013.12.009","article-title":"Neuronal matrix metalloproteinase-9 is a determinant of selective neurodegeneration","volume":"81","author":"A Kaplan","year":"2014","journal-title":"Neuron"},{"issue":"5","key":"ref20","doi-asserted-by":"publisher","first-page":"822","DOI":"10.1086\/425287","article-title":"A mutation in the vesicle-trafficking protein VAPB causes late-onset spinal muscular atrophy and amyotrophic lateral sclerosis","volume":"75","author":"AL Nishimura","year":"2004","journal-title":"Am J Hum Genet"},{"issue":"51","key":"ref21","doi-asserted-by":"publisher","first-page":"40266","DOI":"10.1074\/jbc.M110.161398","article-title":"Characterization of the properties of a novel mutation in VAPB in familial amyotrophic lateral sclerosis","volume":"285","author":"HJ Chen","year":"2010","journal-title":"J Biol Chem"},{"issue":"22","key":"ref22","doi-asserted-by":"publisher","first-page":"6515","DOI":"10.1093\/hmg\/ddv360","article-title":"Vapb\/Amyotrophic lateral sclerosis 8 knock-in mice display slowly progressive motor behavior defects accompanying ER stress and autophagic response","volume":"24","author":"F Larroquette","year":"2015","journal-title":"Hum Mol Genet"},{"issue":"3","key":"ref23","doi-asserted-by":"publisher","first-page":"e1005107","DOI":"10.1371\/journal.pgen.1005107","article-title":"Network analyses reveal novel aspects of ALS pathogenesis","volume":"11","author":"M Sanhueza","year":"2015","journal-title":"PLoS Genet"},{"issue":"9","key":"ref24","doi-asserted-by":"publisher","first-page":"3871","DOI":"10.1091\/mbc.E08-05-0498","article-title":"Coordinated lipid transfer between the endoplasmic reticulum and the Golgi complex requires the VAP proteins and is essential for Golgi-mediated transport","volume":"19","author":"D Peretti","year":"2008","journal-title":"Mol Biol Cell"},{"issue":"3","key":"ref25","doi-asserted-by":"publisher","first-page":"371","DOI":"10.1016\/j.cub.2016.12.038","article-title":"The ER-Mitochondria Tethering Complex VAPB-PTPIP51 Regulates Autophagy","volume":"27","author":"P Gomez-Suaga","year":"2017","journal-title":"Curr Biol"},{"issue":"9","key":"ref26","doi-asserted-by":"publisher","first-page":"e1003738","DOI":"10.1371\/journal.pgen.1003738","article-title":"VAPB\/ALS8 MSP ligands regulate striated muscle energy metabolism critical for adult survival in caenorhabditis elegans","volume":"9","author":"SM Han","year":"2013","journal-title":"PLoS Genet"},{"issue":"3","key":"ref27","doi-asserted-by":"publisher","first-page":"238","DOI":"10.1002\/dvg.1020120309","article-title":"Generating Lineage-Specific Markers to Study Drosophila Development","volume":"12","author":"N Perrimon","year":"1991","journal-title":"Dev Genet"},{"issue":"13","key":"ref28","doi-asserted-by":"publisher","first-page":"2689","DOI":"10.1093\/hmg\/ddt118","article-title":"Increased levels of phosphoinositides cause neurodegeneration in a Drosophila model of amyotrophic lateral sclerosis","volume":"22","author":"S Forrest","year":"2013","journal-title":"Hum Mol Genet"},{"issue":"3","key":"ref29","doi-asserted-by":"publisher","first-page":"275","DOI":"10.1007\/s12031-010-9422-1","article-title":"Alpha-synuclein loss in spinal muscular atrophy","volume":"43","author":"G Acsadi","year":"2011","journal-title":"J Mol Neurosci"},{"issue":"31","key":"ref30","doi-asserted-by":"publisher","first-page":"24065","DOI":"10.1074\/jbc.M002413200","article-title":"Wild-type but not Parkinson&apos;s disease-related ala-53 \u2014&gt; Thr mutant alpha -synuclein protects neuronal cells from apoptotic stimuli","volume":"275","author":"CA da Costa","year":"2000","journal-title":"J Biol Chem"},{"issue":"13","key":"ref31","doi-asserted-by":"publisher","first-page":"11465","DOI":"10.1074\/jbc.M111428200","article-title":"alpha-Synuclein protects against oxidative stress via inactivation of the c-Jun N-terminal kinase stress-signaling pathway in neuronal cells","volume":"277","author":"M Hashimoto","year":"2002","journal-title":"J Biol Chem"},{"issue":"8","key":"ref32","doi-asserted-by":"crossref","first-page":"3095","DOI":"10.1523\/JNEUROSCI.23-08-03095.2003","article-title":"Alpha-synuclein overexpression protects against paraquat-induced neurodegeneration","volume":"23","author":"AB Manning-Bog","year":"2003","journal-title":"J Neurosci"},{"issue":"1\u20132","key":"ref33","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1016\/S0006-8993(00)02215-0","article-title":"Overexpression of human alpha-synuclein causes dopamine neuron death in rat primary culture and immortalized mesencephalon-derived cells","volume":"866","author":"W Zhou","year":"2000","journal-title":"Brain Res"},{"issue":"1","key":"ref34","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1038\/nbt.1515","article-title":"Intravascular AAV9 preferentially targets neonatal neurons and adult astrocytes","volume":"27","author":"KD Foust","year":"2009","journal-title":"Nat Biotechnol"},{"issue":"17","key":"ref35","doi-asserted-by":"publisher","first-page":"4559","DOI":"10.1093\/hmg\/ddu169","article-title":"Defining the therapeutic window in a severe animal model of spinal muscular atrophy","volume":"23","author":"KL Robbins","year":"2014","journal-title":"Hum Mol Genet"},{"key":"ref36","doi-asserted-by":"publisher","first-page":"7","DOI":"10.3389\/fmolb.2016.00007","article-title":"Copy Number Variations in the Survival Motor Neuron Genes: Implications for Spinal Muscular Atrophy and Other Neurodegenerative Diseases","volume":"3","author":"ME Butchbach","year":"2016","journal-title":"Front Mol Biosci"},{"issue":"4","key":"ref37","doi-asserted-by":"publisher","first-page":"1285","DOI":"10.1128\/MCB.21.4.1285-1296.2001","article-title":"The CELF family of RNA binding proteins is implicated in cell-specific and developmentally regulated alternative splicing","volume":"21","author":"AN Ladd","year":"2001","journal-title":"Mol Cell Biol"},{"key":"ref38","doi-asserted-by":"publisher","first-page":"302","DOI":"10.1016\/j.jsbmb.2015.01.002","article-title":"Progesterone attenuates Abeta(25\u201335)-induced neuronal toxicity via JNK inactivation and progesterone receptor membrane component 1-dependent inhibition of mitochondrial apoptotic pathway","volume":"154","author":"Y Qin","year":"2015","journal-title":"J Steroid Biochem Mol Biol"},{"issue":"1","key":"ref39","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1111\/jnc.13388","article-title":"Progesterone receptor signalling in retinal photoreceptor neuroprotection","volume":"136","author":"AC Jackson","year":"2016","journal-title":"J Neurochem"},{"issue":"2","key":"ref40","doi-asserted-by":"publisher","first-page":"242","DOI":"10.1002\/jcb.20187","article-title":"The role of stathmin in the regulation of the cell cycle","volume":"93","author":"CI Rubin","year":"2004","journal-title":"J Cell Biochem"},{"issue":"2","key":"ref41","doi-asserted-by":"publisher","first-page":"469","DOI":"10.1016\/S0002-9440(10)64866-3","article-title":"Stathmin-deficient mice develop an age-dependent axonopathy of the central and peripheral nervous systems","volume":"160","author":"W Liedtke","year":"2002","journal-title":"Am J Pathol"},{"issue":"5","key":"ref42","doi-asserted-by":"publisher","first-page":"1701","DOI":"10.1016\/S0002-9440(10)63426-8","article-title":"Dysregulation of stathmin, a microtubule-destabilizing protein, and up-regulation of Hsp25, Hsp27, and the antioxidant peroxiredoxin 6 in a mouse model of familial amyotrophic lateral sclerosis","volume":"165","author":"CW Strey","year":"2004","journal-title":"Am J Pathol"},{"key":"ref43","doi-asserted-by":"publisher","first-page":"94","DOI":"10.1016\/j.nbd.2012.11.015","article-title":"Decreased stathmin expression ameliorates neuromuscular defects but fails to prolong survival in a mouse model of spinal muscular atrophy","volume":"52","author":"HL Wen","year":"2013","journal-title":"Neurobiol Dis"},{"issue":"5321","key":"ref44","doi-asserted-by":"publisher","first-page":"2045","DOI":"10.1126\/science.276.5321.2045","article-title":"Mutation in the alpha-synuclein gene identified in families with Parkinson&apos;s disease","volume":"276","author":"MH Polymeropoulos","year":"1997","journal-title":"Science"},{"issue":"8","key":"ref45","doi-asserted-by":"crossref","first-page":"2804","DOI":"10.1523\/JNEUROSCI.08-08-02804.1988","article-title":"Synuclein: a neuron-specific protein localized to the nucleus and presynaptic nerve terminal","volume":"8","author":"L Maroteaux","year":"1988","journal-title":"J Neurosci"},{"issue":"1","key":"ref46","doi-asserted-by":"publisher","first-page":"66","DOI":"10.1016\/j.neuron.2009.12.023","article-title":"Increased expression of alpha-synuclein reduces neurotransmitter release by inhibiting synaptic vesicle reclustering after endocytosis","volume":"65","author":"VM Nemani","year":"2010","journal-title":"Neuron"},{"issue":"49","key":"ref47","doi-asserted-by":"publisher","first-page":"11165","DOI":"10.1523\/JNEUROSCI.2559-04.2004","article-title":"Role of alpha-synuclein in presynaptic dopamine recruitment","volume":"24","author":"L Yavich","year":"2004","journal-title":"J Neurosci"},{"issue":"1","key":"ref48","doi-asserted-by":"publisher","first-page":"376","DOI":"10.1016\/j.bbrc.2011.11.121","article-title":"Direct central nervous system delivery provides enhanced protection following vector mediated gene replacement in a severe model of spinal muscular atrophy","volume":"417","author":"JJ Glascock","year":"2012","journal-title":"Biochem Biophys Res Commun"},{"issue":"2","key":"ref49","doi-asserted-by":"publisher","first-page":"135","DOI":"10.1089\/hum.2010.114","article-title":"Combination of SMN trans-splicing and a neurotrophic factor increases the life span and body mass in a severe model of spinal muscular atrophy","volume":"22","author":"M Shababi","year":"2011","journal-title":"Hum Gene Ther"}],"updated-by":[{"DOI":"10.1371\/journal.pgen.1006680","type":"new_version","label":"New version","source":"publisher","updated":{"date-parts":[[2017,4,14]],"date-time":"2017-04-14T00:00:00Z","timestamp":1492128000000}}],"container-title":["PLOS Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pgen.1006680","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,9,20]],"date-time":"2019-09-20T09:30:22Z","timestamp":1568971822000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pgen.1006680"}},"subtitle":[],"editor":[{"given":"Gregory A.","family":"Cox","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2017,3,31]]},"references-count":49,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2017,3,31]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pgen.1006680","relation":{"new_version":[{"id-type":"doi","id":"10.1371\/journal.pgen.1006680","asserted-by":"object"}]},"ISSN":["1553-7404"],"issn-type":[{"value":"1553-7404","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,3,31]]}}}