{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T04:31:59Z","timestamp":1791174719434,"version":"4.1.0"},"reference-count":36,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2009,6,1]],"date-time":"2009-06-01T00:00:00Z","timestamp":1243814400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2009,6,1]],"date-time":"2009-06-01T00:00:00Z","timestamp":1243814400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["bmn.com","clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Current Opinion in Cell Biology"],"published-print":{"date-parts":[[2009,6]]},"DOI":"10.1016\/j.ceb.2009.02.004","type":"journal-article","created":{"date-parts":[[2009,3,14]],"date-time":"2009-03-14T06:41:01Z","timestamp":1237012861000},"page":"387-393","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":86,"title":["The role of RNP biogenesis in spinal muscular atrophy"],"prefix":"10.1016","volume":"21","author":[{"given":"Ashwin","family":"Chari","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elham","family":"Paknia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Utz","family":"Fischer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ceb.2009.02.004_bib1","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1146\/annurev.genet.36.043002.091635","article-title":"Allosteric cascade of spliceosome activation","volume":"36","author":"Brow","year":"2002","journal-title":"Annu Rev Genet"},{"key":"10.1016\/j.ceb.2009.02.004_bib2","doi-asserted-by":"crossref","first-page":"960","DOI":"10.1038\/nrm1259","article-title":"Splicing double: insights from the second spliceosome","volume":"4","author":"Patel","year":"2003","journal-title":"Nat Rev Mol Cell Biol"},{"key":"10.1016\/j.ceb.2009.02.004_bib3","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1016\/S0955-0674(00)00211-8","article-title":"Spliceosomal UsnRNP biogenesis, structure and function","volume":"13","author":"Will","year":"2001","journal-title":"Curr Opin Cell Biol"},{"key":"10.1016\/j.ceb.2009.02.004_bib4","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1038\/nrm1645","article-title":"Understanding alternative splicing: towards a cellular code","volume":"6","author":"Matlin","year":"2005","journal-title":"Nat Rev Mol Cell Biol"},{"key":"10.1016\/j.ceb.2009.02.004_bib5","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1146\/annurev.biochem.72.121801.161720","article-title":"Mechanisms of alternative pre-messenger RNA splicing","volume":"72","author":"Black","year":"2003","journal-title":"Annu Rev Biochem"},{"key":"10.1016\/j.ceb.2009.02.004_bib6","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1038\/nrg775","article-title":"Listening to silence and understanding nonsense: exonic mutations that affect splicing","volume":"3","author":"Cartegni","year":"2002","journal-title":"Nat Rev Genet"},{"key":"10.1016\/j.ceb.2009.02.004_bib7","doi-asserted-by":"crossref","first-page":"926","DOI":"10.1177\/0883073807305662","article-title":"Spinal muscular atrophy: classification, diagnosis, management, pathogenesis, and future research directions","volume":"22","author":"Kostova","year":"2007","journal-title":"J Child Neurol"},{"key":"10.1016\/j.ceb.2009.02.004_bib8","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.ceb.2009.02.004_bib9","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 U S A"},{"key":"10.1016\/j.ceb.2009.02.004_bib10","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.molmed.2006.01.005","article-title":"Spinal muscular atrophy: the RNP connection","volume":"12","author":"Eggert","year":"2006","journal-title":"Trends Mol Med"},{"key":"10.1016\/j.ceb.2009.02.004_bib11","doi-asserted-by":"crossref","first-page":"3629","DOI":"10.1093\/hmg\/ddi390","article-title":"The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiation","volume":"14","author":"Gabanella","year":"2005","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.ceb.2009.02.004_bib12","doi-asserted-by":"crossref","first-page":"1997","DOI":"10.1016\/j.febslet.2008.03.009","article-title":"Deciphering the assembly pathway of Sm-class U snRNPs","volume":"582","author":"Neuenkirchen","year":"2008","journal-title":"FEBS Lett"},{"key":"10.1016\/j.ceb.2009.02.004_bib13","doi-asserted-by":"crossref","first-page":"2256","DOI":"10.1002\/j.1460-2075.1996.tb00579.x","article-title":"The snRNP core assembly pathway: identification of stable core protein heteromeric complexes and an snRNP subcore particle in vitro","volume":"15","author":"Raker","year":"1996","journal-title":"EMBO J"},{"key":"10.1016\/j.ceb.2009.02.004_bib14","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.ceb.2009.02.004_bib15","doi-asserted-by":"crossref","first-page":"8126","DOI":"10.1074\/jbc.M512243200","article-title":"Gemin8 is a novel component of the survival motor neuron complex and functions in small nuclear ribonucleoprotein assembly","volume":"281","author":"Carissimi","year":"2006","journal-title":"J Biol Chem"},{"key":"10.1016\/j.ceb.2009.02.004_bib16","doi-asserted-by":"crossref","first-page":"37009","DOI":"10.1074\/jbc.M607505200","article-title":"Gemin8 is required for the architecture and function of the survival motor neuron complex","volume":"281","author":"Carissimi","year":"2006","journal-title":"J Biol Chem"},{"key":"10.1016\/j.ceb.2009.02.004_bib17","doi-asserted-by":"crossref","first-page":"5825","DOI":"10.1074\/jbc.M608528200","article-title":"A comprehensive interaction map of the human survival of motor neuron (SMN) complex","volume":"282","author":"Otter","year":"2007","journal-title":"J Biol Chem"},{"key":"10.1016\/j.ceb.2009.02.004_bib18","doi-asserted-by":"crossref","first-page":"5543","DOI":"10.1128\/MCB.25.13.5543-5551.2005","article-title":"The survival of motor neurons protein determines the capacity for snRNP assembly: biochemical deficiency in spinal muscular atrophy","volume":"25","author":"Wan","year":"2005","journal-title":"Mol Cell Biol"},{"key":"10.1016\/j.ceb.2009.02.004_bib19","doi-asserted-by":"crossref","first-page":"17372","DOI":"10.1073\/pnas.0508947102","article-title":"Gemin proteins are required for efficient assembly of Sm-class ribonucleoproteins","volume":"102","author":"Shpargel","year":"2005","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1016\/j.ceb.2009.02.004_bib20","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1038\/ncb1101-945","article-title":"A multiprotein complex mediates the ATP-dependent assembly of spliceosomal U snRNPs","volume":"3","author":"Meister","year":"2001","journal-title":"Nat Cell Biol"},{"key":"10.1016\/j.ceb.2009.02.004_bib21_1","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.ceb.2009.02.004_bib22","doi-asserted-by":"crossref","first-page":"8289","DOI":"10.1128\/MCB.21.24.8289-8300.2001","article-title":"The methylosome, a 20S complex containing JBP1 and pICln, produces dimethylarginine-modified Sm proteins","volume":"21","author":"Friesen","year":"2001","journal-title":"Mol Cell Biol"},{"key":"10.1016\/j.ceb.2009.02.004_bib23","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1016\/S0960-9822(01)00592-9","article-title":"Methylation of Sm proteins by a complex containing PRMT5 and the putative U snRNP assembly factor pICln","volume":"11","author":"Meister","year":"2001","journal-title":"Curr Biol"},{"key":"10.1016\/j.ceb.2009.02.004_bib24_1","doi-asserted-by":"crossref","first-page":"5853","DOI":"10.1093\/emboj\/cdf585","article-title":"Assisted RNP assembly: SMN and PRMT5 complexes cooperate in the formation of spliceosomal UsnRNPs","volume":"21","author":"Meister","year":"2002","journal-title":"EMBO J"},{"key":"10.1016\/j.ceb.2009.02.004_bib25_1","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1016\/j.cell.2008.09.020","article-title":"An assembly chaperone collaborates with the SMN complex to generate spliceosomal SnRNPs","volume":"135","author":"Chari","year":"2008","journal-title":"Cell"},{"key":"10.1016\/j.ceb.2009.02.004_bib26","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1016\/0092-8674(85)90271-5","article-title":"Small nuclear ribonucleoprotein particle assembly in vivo: demonstration of a 6S RNA-free core precursor and posttranslational modification","volume":"42","author":"Fisher","year":"1985","journal-title":"Cell"},{"key":"10.1016\/j.ceb.2009.02.004_bib27","doi-asserted-by":"crossref","first-page":"1189","DOI":"10.1016\/j.cell.2007.04.025","article-title":"Structure and function of RbcX, an assembly chaperone for hexadecameric Rubisco","volume":"129","author":"Saschenbrecker","year":"2007","journal-title":"Cell"},{"key":"10.1016\/j.ceb.2009.02.004_bib28","doi-asserted-by":"crossref","first-page":"1296","DOI":"10.1016\/j.str.2008.07.001","article-title":"PACemakers of proteasome core particle assembly","volume":"16","author":"Ramos","year":"2008","journal-title":"Structure"},{"key":"10.1016\/j.ceb.2009.02.004_bib29_1","doi-asserted-by":"crossref","first-page":"10045","DOI":"10.1073\/pnas.0802287105","article-title":"Evolution of an RNP assembly system: a minimal SMN complex facilitates formation of UsnRNPs in Drosophila melanogaster","volume":"105","author":"Kroiss","year":"2008","journal-title":"PNAS"},{"key":"10.1016\/j.ceb.2009.02.004_bib30","doi-asserted-by":"crossref","first-page":"37908","DOI":"10.1074\/jbc.274.53.37908","article-title":"A role for polyproline motifs in the spinal muscular atrophy protein SMN. Profilins bind to and colocalize with smn in nuclear gems","volume":"274","author":"Giesemann","year":"1999","journal-title":"J Biol Chem"},{"key":"10.1016\/j.ceb.2009.02.004_bib31","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1083\/jcb.200304128","article-title":"Smn, the spinal muscular atrophy-determining gene product, modulates axon growth and localization of beta-actin mRNA in growth cones of motoneurons","volume":"163","author":"Rossoll","year":"2003","journal-title":"J Cell Biol"},{"key":"10.1016\/j.ceb.2009.02.004_bib32","doi-asserted-by":"crossref","first-page":"946","DOI":"10.1002\/bies.20283","article-title":"Is spinal muscular atrophy the result of defects in motor neuron processes?","volume":"27","author":"Briese","year":"2005","journal-title":"Bioessays"},{"key":"10.1016\/j.ceb.2009.02.004_bib33","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1016\/j.neuron.2005.12.001","article-title":"Spinal muscular atrophy: a deficiency in a ubiquitous protein; a motor neuron-specific disease","volume":"48","author":"Monani","year":"2005","journal-title":"Neuron"},{"key":"10.1016\/j.ceb.2009.02.004_bib34_1","doi-asserted-by":"crossref","first-page":"2320","DOI":"10.1101\/gad.342005","article-title":"Reduced U snRNP assembly causes motor axon degeneration in an animal model for spinal muscular atrophy","volume":"19","author":"Winkler","year":"2005","journal-title":"Genes Dev"},{"key":"10.1016\/j.ceb.2009.02.004_bib35_1","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1016\/j.cell.2008.03.031","article-title":"SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing","volume":"133","author":"Zhang","year":"2008","journal-title":"Cell"},{"key":"10.1016\/j.ceb.2009.02.004_bib36_1","doi-asserted-by":"crossref","first-page":"e921","DOI":"10.1371\/journal.pone.0000921","article-title":"Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs","volume":"2","author":"Gabanella","year":"2007","journal-title":"PLoS ONE"}],"container-title":["Current Opinion in Cell Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0955067409000490?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0955067409000490?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,16]],"date-time":"2025-10-16T13:08:45Z","timestamp":1760620125000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0955067409000490"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,6]]},"references-count":36,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2009,6]]}},"alternative-id":["S0955067409000490"],"URL":"https:\/\/doi.org\/10.1016\/j.ceb.2009.02.004","relation":{},"ISSN":["0955-0674"],"issn-type":[{"value":"0955-0674","type":"print"}],"subject":[],"published":{"date-parts":[[2009,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"The role of RNP biogenesis in spinal muscular atrophy","name":"articletitle","label":"Article Title"},{"value":"Current Opinion in Cell Biology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ceb.2009.02.004","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2009 Elsevier Ltd. All rights reserved.","name":"copyright","label":"Copyright"}]}}