{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T09:45:52Z","timestamp":1791279952863,"version":"4.1.0"},"reference-count":41,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2007,4,1]],"date-time":"2007-04-01T00:00:00Z","timestamp":1175385600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2007,4,1]],"date-time":"2007-04-01T00:00:00Z","timestamp":1175385600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["EMBO Rep"],"published-print":{"date-parts":[[2007,4,1]]},"DOI":"10.1038\/sj.embor.7400941","type":"journal-article","created":{"date-parts":[[2007,4,2]],"date-time":"2007-04-02T04:39:07Z","timestamp":1175488747000},"page":"340-345","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":165,"title":["Chaperoning ribonucleoprotein biogenesis in health and disease"],"prefix":"10.1038","volume":"8","author":[{"given":"Livio","family":"Pellizzoni","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2007,4,1]]},"reference":[{"key":"BFEMBR7400941_CR1","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1016\/j.molcel.2006.05.036","volume":"23","author":"DJ Battle","year":"2006","unstructured":"Battle DJ, Lau CK, Wan L, Deng H, Lotti F, Dreyfuss G (2006) The Gemin5 protein of the SMN complex identifies snRNAs. Mol Cell 23: 273\u2013279","journal-title":"Mol Cell"},{"key":"BFEMBR7400941_CR2","doi-asserted-by":"crossref","first-page":"2351","DOI":"10.1093\/hmg\/8.13.2351","volume":"8","author":"D Buhler","year":"1999","unstructured":"Buhler D, Raker V, Luhrmann R, Fischer U (1999) Essential role for the tudor domain of SMN in spliceosomal U snRNP assembly: implications for spinal muscular atrophy. Hum Mol Genet 8: 2351\u20132357","journal-title":"Hum Mol Genet"},{"key":"BFEMBR7400941_CR3","doi-asserted-by":"crossref","first-page":"2348","DOI":"10.1016\/j.febslet.2005.03.034","volume":"579","author":"C Carissimi","year":"2005","unstructured":"Carissimi C, Baccon J, Straccia M, Chiarella P, Maiolica A, Sawyer A, Rappsilber J, Pellizzoni L (2005) Unrip is a component of SMN complexes active in snRNP assembly. FEBS Lett 579: 2348\u20132354","journal-title":"FEBS Lett"},{"key":"BFEMBR7400941_CR4","doi-asserted-by":"crossref","first-page":"8126","DOI":"10.1074\/jbc.M512243200","volume":"281","author":"C Carissimi","year":"2006","unstructured":"Carissimi C, Saieva L, Baccon J, Chiarella P, Maiolica A, Sawyer A, Rappsilber J, Pellizzoni L (2006a) Gemin8 is a novel component of the survival motor neuron complex and functions in snRNP assembly. J Biol Chem 281: 8126\u20138134","journal-title":"J Biol Chem"},{"key":"BFEMBR7400941_CR5","doi-asserted-by":"crossref","first-page":"37009","DOI":"10.1074\/jbc.M607505200","volume":"281","author":"C Carissimi","year":"2006","unstructured":"Carissimi C, Saieva L, Gabanella F, Pellizzoni L (2006b) Gemin8 is required for the architecture and function of the survival motor neuron complex. J Biol Chem 281: 37009\u201337016","journal-title":"J Biol Chem"},{"key":"BFEMBR7400941_CR6","doi-asserted-by":"crossref","first-page":"11014","DOI":"10.1523\/JNEUROSCI.1637-06.2006","volume":"26","author":"TL Carrel","year":"2006","unstructured":"Carrel TL, McWhorter ML, Workman E, Zhang H, Wolstencroft EC, Lorson C, Bassell GJ, Burghes AH, Beattie CE (2006) Survival motor neuron function in motor axons is independent of functions required for small nuclear ribonucleoprotein biogenesis. J Neurosci 26: 11014\u201311022","journal-title":"J Neurosci"},{"key":"BFEMBR7400941_CR7","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1083\/jcb.147.4.715","volume":"147","author":"T Carvalho","year":"1999","unstructured":"Carvalho T, Almeida F, Calapez A, Lafarga M, Berciano MT, Carmo\u2010Fonseca M (1999) The spinal muscular atrophy disease gene product, SMN: A link between snRNP biogenesis and the Cajal (coiled) body. J Cell Biol 147: 715\u2013728","journal-title":"J Cell Biol"},{"key":"BFEMBR7400941_CR8","doi-asserted-by":"crossref","first-page":"1605","DOI":"10.1093\/hmg\/ddi168","volume":"14","author":"W Feng","year":"2005","unstructured":"Feng W, Gubitz AK, Wan L, Battle DJ, Dostie J, Golembe TJ, Dreyfuss G (2005) Gemins modulate the expression and activity of the SMN complex. Hum Mol Genet 14: 1605\u20131611","journal-title":"Hum Mol Genet"},{"key":"BFEMBR7400941_CR9","doi-asserted-by":"crossref","first-page":"3629","DOI":"10.1093\/hmg\/ddi390","volume":"14","author":"F Gabanella","year":"2005","unstructured":"Gabanella F, Carissimi C, Usiello A, Pellizzoni L (2005) The activity of the spinal muscular atrophy protein is regulated during development and cellular differentiation. Hum Mol Genet 14: 3629\u20133642","journal-title":"Hum Mol Genet"},{"key":"BFEMBR7400941_CR10","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1097\/01.jnen.0000205144.54457.a3","volume":"65","author":"A Giavazzi","year":"2006","unstructured":"Giavazzi A, Setola V, Simonati A, Battaglia G (2006) Neuronal\u2010specific roles of the survival motor neuron protein: evidence from survival motor neuron expression patterns in the developing human central nervous system. J Neuropathol Exp Neurol 65: 267\u2013277","journal-title":"J Neuropathol Exp Neurol"},{"key":"BFEMBR7400941_CR11","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1128\/MCB.25.2.602-611.2005","volume":"25","author":"TJ Golembe","year":"2005","unstructured":"Golembe TJ, Yong J, Battle DJ, Feng W, Wan L, Dreyfuss G (2005a) Lymphotropic herpesvirus saimiri uses the SMN complex to assemble Sm cores on its small RNAs. Mol Cell Biol 25: 602\u2013611","journal-title":"Mol Cell Biol"},{"key":"BFEMBR7400941_CR12","doi-asserted-by":"crossref","first-page":"10989","DOI":"10.1128\/MCB.25.24.10989-11004.2005","volume":"25","author":"TJ Golembe","year":"2005","unstructured":"Golembe TJ, Yong J, Dreyfuss G (2005b) Specific sequence features, recognized by the SMN complex, identify snRNAs and determine their fate as snRNPs. Mol Cell Biol 25: 10989\u201311004","journal-title":"Mol Cell Biol"},{"key":"BFEMBR7400941_CR13","doi-asserted-by":"crossref","first-page":"1077","DOI":"10.1016\/j.cub.2006.04.037","volume":"16","author":"GB Gonsalvez","year":"2006","unstructured":"Gonsalvez GB, Rajendra TK, Tian L, Matera AG (2006) The Sm\u2010protein methyltransferase, dart5, is essential for germ\u2010cell specification and maintenance. Curr Biol 16: 1077\u20131089","journal-title":"Curr Biol"},{"key":"BFEMBR7400941_CR14","doi-asserted-by":"crossref","first-page":"3099","DOI":"10.1093\/hmg\/ddi343","volume":"14","author":"M Grimmler","year":"2005","unstructured":"Grimmler M, Otter S, Peter C, Muller F, Chari A, Fischer U (2005) Unrip, a factor implicated in cap\u2010independent translation, associates with the cytosolic SMN complex and influences its intracellular localization. Hum Mol Genet 14: 3099\u20133111","journal-title":"Hum Mol Genet"},{"key":"BFEMBR7400941_CR15","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1016\/S1534-5807(02)00222-8","volume":"3","author":"MD Hebert","year":"2002","unstructured":"Hebert MD, Shpargel KB, Ospina JK, Tucker KE, Matera AG (2002) Coilin methylation regulates nuclear body formation. Dev Cell 3: 329\u2013337","journal-title":"Dev Cell"},{"key":"BFEMBR7400941_CR16","doi-asserted-by":"crossref","first-page":"3555","DOI":"10.1002\/j.1460-2075.1996.tb00725.x","volume":"15","author":"Q Liu","year":"1996","unstructured":"Liu Q, Dreyfuss G (1996) A novel nuclear structure containing the survival of motor neurons protein. EMBO J 15: 3555\u20133565","journal-title":"EMBO J"},{"key":"BFEMBR7400941_CR17","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1038\/ng0598-63","volume":"19","author":"CL Lorson","year":"1998","unstructured":"Lorson CL, Strasswimmer J, Yao JM, Baleja JD, Hahnen E, Wirth B, Le T, Burghes AH, Androphy EJ (1998) SMN oligomerization defect correlates with spinal muscular atrophy severity. Nat Genet 19: 63\u201366","journal-title":"Nat Genet"},{"key":"BFEMBR7400941_CR18","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1016\/j.str.2005.03.014","volume":"13","author":"Y Ma","year":"2005","unstructured":"Ma Y, Dostie J, Dreyfuss G, Van Duyne GD (2005) The Gemin6\u2013Gemin7 heterodimer from the survival of motor neurons complex has an Sm protein\u2010like structure. Structure 13: 883\u2013889","journal-title":"Structure"},{"key":"BFEMBR7400941_CR19","doi-asserted-by":"crossref","first-page":"5853","DOI":"10.1093\/emboj\/cdf585","volume":"21","author":"G Meister","year":"2002","unstructured":"Meister G, Fischer U (2002) Assisted RNP assembly: SMN and PRMT5 complexes cooperate in the formation of spliceosomal UsnRNPs. EMBO J 21: 5853\u20135863","journal-title":"EMBO J"},{"key":"BFEMBR7400941_CR20","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1038\/ncb1101-945","volume":"3","author":"G Meister","year":"2001","unstructured":"Meister G, Buhler D, Pillai R, Lottspeich F, Fischer U (2001) A multiprotein complex mediates the ATP\u2010dependent assembly of spliceosomal U snRNPs. Nat Cell Biol 3: 945\u2013949","journal-title":"Nat Cell Biol"},{"key":"BFEMBR7400941_CR21","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1016\/S0962-8924(02)02371-1","volume":"12","author":"G Meister","year":"2002","unstructured":"Meister G, Eggert C, Fischer U (2002) SMN\u2010mediated assembly of RNPs: a complex story. Trends Cell Biol 12: 472\u2013478","journal-title":"Trends Cell Biol"},{"key":"BFEMBR7400941_CR22","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1016\/j.neuron.2005.12.001","volume":"48","author":"UR Monani","year":"2005","unstructured":"Monani UR (2005) Spinal muscular atrophy: a deficiency in a ubiquitous protein; a motor neuron\u2010specific disease. Neuron 48: 885\u2013896","journal-title":"Neuron"},{"key":"BFEMBR7400941_CR23","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1038\/sj.embor.embor863","volume":"4","author":"J Mouaikel","year":"2003","unstructured":"Mouaikel J, Narayanan U, Verheggen C, Matera AG, Bertrand E, Tazi J, Bordonne R (2003) Interaction between the small\u2010nuclear\u2010RNA cap hypermethylase and the spinal muscular atrophy protein, survival of motor neuron. EMBO Rep 4: 616\u2013622","journal-title":"EMBO Rep"},{"key":"BFEMBR7400941_CR24","doi-asserted-by":"crossref","first-page":"720","DOI":"10.1101\/gad.974702","volume":"16","author":"Z Mourelatos","year":"2002","unstructured":"Mourelatos Z, Dostie J, Paushkin S, Sharma A, Charroux B, Abel L, Rappsilber J, Mann M, Dreyfuss G (2002) miRNPs: a novel class of ribonucleoproteins containing numerous microRNAs. Genes Dev 16: 720\u2013728","journal-title":"Genes Dev"},{"key":"BFEMBR7400941_CR25","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1016\/j.molcel.2004.09.024","volume":"16","author":"U Narayanan","year":"2004","unstructured":"Narayanan U, Achsel T, Luhrmann R, Matera AG (2004) Coupled in vitro import of U snRNPs and SMN, the spinal muscular atrophy protein. Mol Cell 16: 223\u2013234","journal-title":"Mol Cell"},{"key":"BFEMBR7400941_CR26","doi-asserted-by":"crossref","first-page":"5825","DOI":"10.1074\/jbc.M608528200","volume":"282","author":"S Otter","year":"2007","unstructured":"Otter S, Grimmler M, Neuenkirchen N, Chari A, Sickmann A, Fischer U (2007) A comprehensive interaction map of the human SMN\u2013complex. J Biol Chem 282: 5825\u20135833","journal-title":"J Biol Chem"},{"key":"BFEMBR7400941_CR27","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1016\/S0955-0674(02)00332-0","volume":"14","author":"S Paushkin","year":"2002","unstructured":"Paushkin S, Gubitz AK, Massenet S, Dreyfuss G (2002) The SMN complex, an assemblyosome of ribonucleoproteins. Curr Opin Cell Biol 14: 305\u2013312","journal-title":"Curr Opin Cell Biol"},{"key":"BFEMBR7400941_CR28","doi-asserted-by":"crossref","first-page":"11167","DOI":"10.1073\/pnas.96.20.11167","volume":"96","author":"L Pellizzoni","year":"1999","unstructured":"Pellizzoni L, Charroux B, Dreyfuss G (1999) SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins. Proc Natl Acad Sci USA 96: 11167\u201311172","journal-title":"Proc Natl Acad Sci USA"},{"key":"BFEMBR7400941_CR29","doi-asserted-by":"crossref","first-page":"1775","DOI":"10.1126\/science.1074962","volume":"298","author":"L Pellizzoni","year":"2002","unstructured":"Pellizzoni L, Yong J, Dreyfuss G (2002) Essential role for the SMN complex in the specificity of snRNP assembly. Science 298: 1775\u20131779","journal-title":"Science"},{"key":"BFEMBR7400941_CR30","doi-asserted-by":"crossref","first-page":"2321","DOI":"10.1101\/gad.274403","volume":"17","author":"RS Pillai","year":"2003","unstructured":"Pillai RS, Grimmler M, Meister G, Will CL, Luhrmann R, Fischer U, Schumperli D (2003) Unique Sm core structure of U7 snRNPs: assembly by a specialized SMN complex and the role of a new component, Lsm11, in histone RNA processing. Genes Dev 17: 2321\u20132333","journal-title":"Genes Dev"},{"key":"BFEMBR7400941_CR31","doi-asserted-by":"crossref","first-page":"6554","DOI":"10.1128\/MCB.19.10.6554","volume":"19","author":"VA Raker","year":"1999","unstructured":"Raker VA, Hartmuth K, Kastner B, Luhrmann R (1999) Spliceosomal U snRNP core assembly: Sm proteins assemble onto an Sm site RNA nonanucleotide in a specific and thermodynamically stable manner. Mol Cell Biol 19: 6554\u20136565","journal-title":"Mol Cell Biol"},{"key":"BFEMBR7400941_CR32","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1083\/jcb.200304128","volume":"163","author":"W Rossoll","year":"2003","unstructured":"Rossoll W, Jablonka S, Andreassi C, Kroning AK, Karle K, Monani UR, Sendtner M (2003) Smn, the spinal muscular atrophy\u2010determining gene product, modulates axon growth and localization of beta\u2010actin mRNA in growth cones of motoneurons. J Cell Biol 163: 801\u2013812","journal-title":"J Cell Biol"},{"key":"BFEMBR7400941_CR33","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/j.yexcr.2005.05.014","volume":"309","author":"A Sharma","year":"2005","unstructured":"Sharma A, Lambrechts A, Hao le T, Le TT, Sewry CA, Ampe C, Burghes AH, Morris GE (2005) A role for complexes of survival of motor neurons (SMN) protein with gemins and profilin in neurite\u2010like cytoplasmic extensions of cultured nerve cells. Exp Cell Res 309: 185\u2013197","journal-title":"Exp Cell Res"},{"key":"BFEMBR7400941_CR34","doi-asserted-by":"crossref","first-page":"17372","DOI":"10.1073\/pnas.0508947102","volume":"102","author":"KB Shpargel","year":"2005","unstructured":"Shpargel KB, Matera AG (2005) Gemin proteins are required for efficient assembly of Sm\u2010class ribonucleoproteins. Proc Natl Acad Sci USA 102: 17372\u201317377","journal-title":"Proc Natl Acad Sci USA"},{"key":"BFEMBR7400941_CR35","doi-asserted-by":"crossref","first-page":"5543","DOI":"10.1128\/MCB.25.13.5543-5551.2005","volume":"25","author":"L Wan","year":"2005","unstructured":"Wan L, Battle DJ, Yong J, Gubitz AK, Kolb SJ, Wang J, Dreyfuss G (2005) The survival of motor neurons protein determines the capacity for snRNP assembly: biochemical deficiency in spinal muscular atrophy. Mol Cell Biol 25: 5543\u20135551","journal-title":"Mol Cell Biol"},{"key":"BFEMBR7400941_CR36","doi-asserted-by":"crossref","first-page":"290","DOI":"10.1016\/S0955-0674(00)00211-8","volume":"13","author":"CL Will","year":"2001","unstructured":"Will CL, Luhrmann R (2001) Spliceosomal UsnRNP biogenesis, structure and function. Curr Opin Cell Biol 13: 290\u2013301","journal-title":"Curr Opin Cell Biol"},{"key":"BFEMBR7400941_CR37","doi-asserted-by":"crossref","first-page":"2320","DOI":"10.1101\/gad.342005","volume":"19","author":"C Winkler","year":"2005","unstructured":"Winkler C, Eggert C, Gradl D, Meister G, Giegerich M, Wedlich D, Laggerbauer B, Fischer U (2005) Reduced U snRNP assembly causes motor axon degeneration in an animal model for spinal muscular atrophy. Genes Dev 19: 2320\u20132330","journal-title":"Genes Dev"},{"key":"BFEMBR7400941_CR38","doi-asserted-by":"crossref","first-page":"1188","DOI":"10.1093\/emboj\/21.5.1188","volume":"21","author":"J Yong","year":"2002","unstructured":"Yong J, Pellizzoni L, Dreyfuss G (2002) Sequence\u2010specific interaction of U1 snRNA with the SMN complex. EMBO J 21: 1188\u20131196","journal-title":"EMBO J"},{"key":"BFEMBR7400941_CR39","doi-asserted-by":"crossref","first-page":"2747","DOI":"10.1128\/MCB.24.7.2747-2756.2004","volume":"24","author":"J Yong","year":"2004","unstructured":"Yong J, Golembe TJ, Battle DJ, Pellizzoni L, Dreyfuss G (2004) snRNAs contain specific SMN\u2010binding domains that are essential for snRNP assembly. Mol Cell Biol 24: 2747\u20132756","journal-title":"Mol Cell Biol"},{"key":"BFEMBR7400941_CR40","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1006\/excr.2001.5186","volume":"265","author":"PJ Young","year":"2001","unstructured":"Young PJ, Le TT, Dunckley M, Nguyen TM, Burghes AH, Morris GE (2001) Nuclear gems and Cajal (coiled) bodies in fetal tissues: nucleolar distribution of the spinal muscular atrophy protein, SMN. Exp Cell Res 265: 252\u2013261","journal-title":"Exp Cell Res"},{"key":"BFEMBR7400941_CR41","doi-asserted-by":"crossref","first-page":"8622","DOI":"10.1523\/JNEUROSCI.3967-05.2006","volume":"26","author":"H Zhang","year":"2006","unstructured":"Zhang H, Xing L, Rossoll W, Wichterle H, Singer RH, Bassell GJ (2006) Multiprotein complexes of the survival of motor neuron protein SMN with Gemins traffic to neuronal processes and growth cones of motor neurons. J Neurosci 26: 8622\u20138632","journal-title":"J Neurosci"}],"container-title":["The EMBO Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1038%2Fsj.embor.7400941","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1038\/sj.embor.7400941.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1038\/sj.embor.7400941","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1038\/sj.embor.7400941\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.embopress.org\/doi\/pdf\/10.1038\/sj.embor.7400941","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1038\/sj.embor.7400941.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T20:38:24Z","timestamp":1779136704000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1038\/sj.embor.7400941"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,4,1]]},"references-count":41,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2007,4,1]]}},"alternative-id":["BFEMBR7400941"],"URL":"https:\/\/doi.org\/10.1038\/sj.embor.7400941","relation":{},"ISSN":["1469-221X","1469-3178"],"issn-type":[{"value":"1469-221X","type":"print"},{"value":"1469-3178","type":"electronic"}],"subject":[],"published":{"date-parts":[[2007,4,1]]},"assertion":[{"value":"27 September 2006","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"31 January 2007","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"1 April 2007","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"This content has been made available to all.","name":"free","label":"Free to read"}]}}