{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T12:16:07Z","timestamp":1790943367403,"version":"4.1.0"},"reference-count":32,"publisher":"Elsevier BV","issue":"11","license":[{"start":{"date-parts":[[2004,11,1]],"date-time":"2004-11-01T00:00:00Z","timestamp":1099267200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2004,11,1]],"date-time":"2004-11-01T00:00:00Z","timestamp":1099267200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","nmd-journal.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neuromuscular Disorders"],"published-print":{"date-parts":[[2004,11]]},"DOI":"10.1016\/j.nmd.2004.08.009","type":"journal-article","created":{"date-parts":[[2004,9,28]],"date-time":"2004-09-28T15:29:25Z","timestamp":1096385365000},"page":"711-722","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":22,"title":["Expression profiling in spinal muscular atrophy reveals an RNA binding protein deficit"],"prefix":"10.1016","volume":"14","author":[{"given":"Kirstie N.","family":"Anderson","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dilair","family":"Baban","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter L.","family":"Oliver","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allyson","family":"Potter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kay E.","family":"Davies","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.nmd.2004.08.009_bib1","doi-asserted-by":"crossref","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":"Lefebvre","year":"1995","journal-title":"Cell"},{"key":"10.1016\/j.nmd.2004.08.009_bib2","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1101\/gr.7.4.339","article-title":"Cloning, characterization, and copy number of the murine survival motor neuron gene: homolog of the spinal muscular atrophy-determining gene","volume":"7","author":"DiDonato","year":"1997","journal-title":"Genome Res"},{"key":"10.1016\/j.nmd.2004.08.009_bib3","doi-asserted-by":"crossref","first-page":"6307","DOI":"10.1073\/pnas.96.11.6307","article-title":"A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy","volume":"96","author":"Lorson","year":"1999","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/j.nmd.2004.08.009_bib4","doi-asserted-by":"crossref","first-page":"1205","DOI":"10.1093\/hmg\/6.8.1205","article-title":"The survival motor neuron protein in spinal muscular atrophy","volume":"6","author":"Coovert","year":"1997","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.nmd.2004.08.009_bib5","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1038\/ng0797-265","article-title":"Correlation between severity and SMN protein level in spinal muscular atrophy","volume":"16","author":"Lefebvre","year":"1997","journal-title":"Nat Genet"},{"key":"10.1016\/j.nmd.2004.08.009_bib6","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1006\/excr.2000.4858","article-title":"The relationship between SMN, the spinal muscular atrophy protein, and nuclear coiled bodies in differentiated tissues and cultured cells","volume":"256","author":"Young","year":"2000","journal-title":"Exp Cell Res"},{"key":"10.1016\/j.nmd.2004.08.009_bib7","doi-asserted-by":"crossref","first-page":"3555","DOI":"10.1002\/j.1460-2075.1996.tb00725.x","article-title":"A novel nuclear structure containing the survival of motor neurons protein","volume":"15","author":"Liu","year":"1996","journal-title":"Eur Mol Biol Org J"},{"key":"10.1016\/j.nmd.2004.08.009_bib8","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1038\/ng0598-63","article-title":"SMN oligomerization defect correlates with spinal muscular atrophy severity","volume":"19","author":"Lorson","year":"1998","journal-title":"Nat Genet"},{"key":"10.1016\/j.nmd.2004.08.009_bib9","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.1016\/S0092-8674(00)80368-2","article-title":"The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis","volume":"90","author":"Fischer","year":"1997","journal-title":"Cell"},{"key":"10.1016\/j.nmd.2004.08.009_bib10","doi-asserted-by":"crossref","first-page":"38645","DOI":"10.1074\/jbc.M106161200","article-title":"Direct interaction of the spinal muscular atrophy disease protein SMN with the small nucleolar RNA-associated protein fibrillarin","volume":"276","author":"Jones","year":"2001","journal-title":"J Biol Chem"},{"key":"10.1016\/j.nmd.2004.08.009_bib11","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1083\/jcb.152.1.75","article-title":"A functional interaction between the survival motor neuron complex and RNA polymerase II","volume":"152","author":"Pellizzoni","year":"2001","journal-title":"J Cell Biol"},{"key":"10.1016\/j.nmd.2004.08.009_bib12","doi-asserted-by":"crossref","first-page":"1775","DOI":"10.1126\/science.1074962","article-title":"Essential role for the SMN complex in the specificity of snRNP assembly","volume":"298","author":"Pellizzoni","year":"2002","journal-title":"Science"},{"key":"10.1016\/j.nmd.2004.08.009_bib13","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1016\/S0955-0674(02)00332-0","article-title":"The SMN complex, an assemblyosome of ribonucleoproteins","volume":"14","author":"Paushkin","year":"2002","journal-title":"Curr Opin Cell Biol"},{"key":"10.1016\/j.nmd.2004.08.009_bib14","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1016\/S0962-8924(02)02371-1","article-title":"SMN-mediated assembly of RNPs: a complex story","volume":"12","author":"Meister","year":"2002","journal-title":"Trends Cell Biol"},{"key":"10.1016\/j.nmd.2004.08.009_bib15","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1038\/37144","article-title":"Synergistic anti-apoptotic activity between Bcl-2 and SMN implicated in spinal muscular atrophy","volume":"390","author":"Iwahashi","year":"1997","journal-title":"Nature"},{"key":"10.1016\/j.nmd.2004.08.009_bib16","doi-asserted-by":"crossref","first-page":"2852","DOI":"10.1074\/jbc.M108769200","article-title":"A direct interaction between the survival motor neuron protein and p53 and its relationship to spinal muscular atrophy","volume":"277","author":"Young","year":"2002","journal-title":"J Biol Chem"},{"key":"10.1016\/j.nmd.2004.08.009_bib17","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1038\/35070059","article-title":"Spinal muscular atrophy disrupts the interaction of ZPR1 with the SMN protein","volume":"3","author":"Gangwani","year":"2001","journal-title":"Nat Cell Biol"},{"key":"10.1016\/j.nmd.2004.08.009_bib18","doi-asserted-by":"crossref","first-page":"6627","DOI":"10.1523\/JNEUROSCI.23-16-06627.2003","article-title":"Active transport of the survival motor neuron protein and the role of exon-7 in cytoplasmic localization","volume":"23","author":"Zhang","year":"2003","journal-title":"J Neurosci"},{"key":"10.1016\/j.nmd.2004.08.009_bib19","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1083\/jcb.200303168","article-title":"Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding","volume":"162","author":"McWhorter","year":"2003","journal-title":"J Cell Biol"},{"key":"10.1016\/j.nmd.2004.08.009_bib20","doi-asserted-by":"crossref","first-page":"1367","DOI":"10.1093\/hmg\/ddg157","article-title":"Neuromuscular defects in a Drosophila survival motor neuron gene mutant","volume":"12","author":"Chan","year":"2003","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.nmd.2004.08.009_bib21","doi-asserted-by":"crossref","first-page":"9920","DOI":"10.1073\/pnas.94.18.9920","article-title":"Inactivation of the survival motor neuron gene, a candidate gene for human spinal muscular atrophy, leads to massive cell death in early mouse embryos","volume":"94","author":"Schrank","year":"1997","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/j.nmd.2004.08.009_bib22","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1038\/71709","article-title":"A mouse model for spinal muscular atrophy","volume":"24","author":"Hsieh-Li","year":"2000","journal-title":"Nat Genet"},{"key":"10.1016\/j.nmd.2004.08.009_bib23","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1093\/hmg\/9.3.333","article-title":"The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn(\u2212\/\u2212) mice and results in a mouse with spinal muscular atrophy","volume":"9","author":"Monani","year":"2000","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.nmd.2004.08.009_bib24","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1093\/hmg\/9.3.341","article-title":"Reduced survival motor neuron (Smn) gene dose in mice leads to motor neuron degeneration: an animal model for spinal muscular atrophy type III","volume":"9","author":"Jablonka","year":"2000","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.nmd.2004.08.009_bib25","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1093\/hmg\/11.1.93","article-title":"Specific interaction of Smn, the spinal muscular atrophy determining gene product, with hnRNP-R and gry-rbp\/hnRNP-Q: a role for Smn in RNA processing in motor axons?","volume":"11","author":"Rossoll","year":"2002","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.nmd.2004.08.009_bib26","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1242\/dev.116.1.227","article-title":"Cloning and developmental expression of Sna, a murine homologue of the drosophila snail gene","volume":"116","author":"Nieto","year":"1992","journal-title":"Development"},{"key":"10.1016\/j.nmd.2004.08.009_bib27","doi-asserted-by":"crossref","first-page":"1895","DOI":"10.1046\/j.1471-4159.2000.0741895.x","article-title":"Development and characterisation of a glutamate-sensitive motor neurone cell line","volume":"74","author":"Eggett","year":"2000","journal-title":"J Neurochem"},{"key":"10.1016\/j.nmd.2004.08.009_bib28","first-page":"961","article-title":"The dsRNA binding protein family: critical roles, diverse cellular functions","volume":"17","author":"Saunders","year":"2003","journal-title":"Fed Am Soc Exp Biol J"},{"key":"10.1016\/j.nmd.2004.08.009_bib29","doi-asserted-by":"crossref","first-page":"1013","DOI":"10.1016\/S0896-6273(01)00551-7","article-title":"Chemotropic responses of retinal growth cones mediated by rapid local protein synthesis and degradation","volume":"32","author":"Campbell","year":"2001","journal-title":"Neuron"},{"key":"10.1016\/j.nmd.2004.08.009_bib30","doi-asserted-by":"crossref","first-page":"28583","DOI":"10.1074\/jbc.M003083200","article-title":"A family of human RNA-binding proteins related to the Drosophila Bruno translational regulator","volume":"275","author":"Good","year":"2000","journal-title":"J Biol Chem"},{"key":"10.1016\/j.nmd.2004.08.009_bib31","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1016\/S0962-8924(00)01767-0","article-title":"Staufen: a common component of mRNA transport in oocytes and neurons?","volume":"10","author":"Roegiers","year":"2000","journal-title":"Trends Cell Biol"},{"key":"10.1016\/j.nmd.2004.08.009_bib32","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1016\/S0140-6736(80)90847-8","article-title":"Classification of spinal muscular atrophies","volume":"1","author":"Pearn","year":"1980","journal-title":"Lancet"}],"container-title":["Neuromuscular Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0960896604002330?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0960896604002330?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,18]],"date-time":"2025-10-18T07:52:35Z","timestamp":1760773955000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0960896604002330"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,11]]},"references-count":32,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2004,11]]}},"alternative-id":["S0960896604002330"],"URL":"https:\/\/doi.org\/10.1016\/j.nmd.2004.08.009","relation":{},"ISSN":["0960-8966"],"issn-type":[{"value":"0960-8966","type":"print"}],"subject":[],"published":{"date-parts":[[2004,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Expression profiling in spinal muscular atrophy reveals an RNA binding protein deficit","name":"articletitle","label":"Article Title"},{"value":"Neuromuscular Disorders","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nmd.2004.08.009","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2004 Published by Elsevier B.V.","name":"copyright","label":"Copyright"}]}}