{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T19:36:05Z","timestamp":1790537765413,"version":"4.1.0"},"reference-count":37,"publisher":"The Company of Biologists","issue":"6","license":[{"start":{"date-parts":[[2020,9,2]],"date-time":"2020-09-02T00:00:00Z","timestamp":1599004800000},"content-version":"vor","delay-in-days":1563,"URL":"http:\/\/creativecommons.org\/licenses\/by\/3.0"}],"funder":[{"DOI":"10.13039\/100006940","name":"University of Mississippi","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006940","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2016,6,15]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Telomerase is a ribonucleoprotein comprising telomerase RNA and associated proteins. The formation of the telomerase holoenzyme takes place in the Cajal body (CB), a subnuclear domain that participates in the formation of ribonucleoproteins. CBs also contribute to the delivery of telomerase to telomeres. The protein WRAP53 is enriched within the CB and is instrumental for the targeting of telomerase RNA to CBs. Two other CB proteins, SMN and coilin, are also suspected of taking part in some aspect of telomerase biogenesis. Here we demonstrate newly discovered associations between SMN and coilin with telomerase components, and further show that reduction of SMN or coilin is correlated with increased association of telomerase RNA with one these components, dyskerin. These findings argue that SMN and coilin may negatively regulate the formation of telomerase. Furthermore, clinically defined SMN mutants found in individuals with spinal muscular atrophy are altered in their association with telomerase complex proteins. Additionally, we observe that a coilin derivative also associates with dyskerin, and the amount of this protein in the complex is regulated by SMN, WRAP53 and coilin levels. Collectively, our findings bolster the link between SMN, coilin and the coilin derivative in the biogenesis of telomerase.<\/jats:p>","DOI":"10.1242\/bio.018804","type":"journal-article","created":{"date-parts":[[2016,5,24]],"date-time":"2016-05-24T04:14:49Z","timestamp":1464063289000},"page":"726-735","source":"Crossref","is-referenced-by-count":31,"title":["SMN and coilin negatively regulate dyskerin association with telomerase RNA"],"prefix":"10.1242","volume":"5","author":[{"given":"Aaron R.","family":"Poole","sequence":"first","affiliation":[{"name":"Department of Biochemistry, The University of Mississippi Medical Center, Jackson, MS 39216-4505, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6233-9654","authenticated-orcid":false,"given":"Michael D.","family":"Hebert","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, The University of Mississippi Medical Center, Jackson, MS 39216-4505, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2016,5,23]]},"reference":[{"key":"2021042423185491300_BIO018804C1","doi-asserted-by":"publisher","first-page":"1947","DOI":"10.1073\/pnas.90.5.1947","article-title":"Immunocytochemical analysis of the coiled body in the cell cycle and during cell proliferation","volume":"90","author":"Andrade","year":"1993","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2021042423185491300_BIO018804C2","doi-asserted-by":"publisher","first-page":"3192","DOI":"10.1091\/mbc.E02-04-0216","article-title":"The product of the survival of motor neuron (SMN) gene is a human telomerase-associated protein","volume":"13","author":"Bachand","year":"2002","journal-title":"Mol. Biol. Cell"},{"key":"2021042423185491300_BIO018804C3","doi-asserted-by":"publisher","first-page":"775","DOI":"10.1093\/hmg\/8.5.775","article-title":"The RNA-binding properties of SMN: deletion analysis of the zebrafish orthologue defines domains conserved in evolution","volume":"8","author":"Bertrandy","year":"1999","journal-title":"Hum. Mol. Genet."},{"key":"2021042423185491300_BIO018804C4","doi-asserted-by":"publisher","first-page":"e36300","DOI":"10.1371\/journal.pone.0036300","article-title":"In vitro RNase and nucleic acid binding activities implicate coilin in U snRNA processing","volume":"7","author":"Broome","year":"2012","journal-title":"PLoS ONE"},{"key":"2021042423185491300_BIO018804C5","doi-asserted-by":"publisher","first-page":"713","DOI":"10.1016\/j.jmb.2012.12.014","article-title":"Coilin displays differential affinity for specific RNAs in vivo and is linked to telomerase RNA biogenesis","volume":"425","author":"Broome","year":"2013","journal-title":"J. Mol. Biol."},{"key":"2021042423185491300_BIO018804C6","doi-asserted-by":"publisher","first-page":"407","DOI":"10.1242\/bio.20133863","article-title":"Phosphorylation regulates coilin activity and RNA association","volume":"2","author":"Broome","year":"2013","journal-title":"Biol. Open"},{"key":"2021042423185491300_BIO018804C7","doi-asserted-by":"publisher","first-page":"597","DOI":"10.1038\/nrn2670","article-title":"Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?","volume":"10","author":"Burghes","year":"2009","journal-title":"Nat. Rev. Neurosci."},{"key":"2021042423185491300_BIO018804C8","doi-asserted-by":"publisher","first-page":"e25743","DOI":"10.1371\/journal.pone.0025743","article-title":"Coilin phosphomutants disrupt Cajal body formation, reduce cell proliferation and produce a distinct coilin degradation product","volume":"6","author":"Carrero","year":"2011","journal-title":"PLoS ONE"},{"key":"2021042423185491300_BIO018804C9","doi-asserted-by":"publisher","first-page":"4462","DOI":"10.1093\/nar\/22.21.4462","article-title":"Structure, expression and chromosomal localization of human p80-coilin gene","volume":"22","author":"Chan","year":"1994","journal-title":"Nucleic Acids Res."},{"key":"2021042423185491300_BIO018804C10","doi-asserted-by":"publisher","first-page":"385","DOI":"10.1093\/nar\/gku1277","article-title":"Human cells lacking coilin and Cajal bodies are proficient in telomerase assembly, trafficking and telomere maintenance","volume":"43","author":"Chen","year":"2015","journal-title":"Nucleic Acids Res."},{"key":"2021042423185491300_BIO018804C11","doi-asserted-by":"publisher","first-page":"546","DOI":"10.1002\/wrna.76","article-title":"SMN in spinal muscular atrophy and snRNP biogenesis","volume":"2","author":"Coady","year":"2011","journal-title":"Wiley Interdiscip. Rev. RNA"},{"key":"2021042423185491300_BIO018804C12","doi-asserted-by":"publisher","first-page":"1747","DOI":"10.1261\/rna.034629.112","article-title":"Biogenesis of telomerase ribonucleoproteins","volume":"18","author":"Egan","year":"2012","journal-title":"RNA"},{"key":"2021042423185491300_BIO018804C13","doi-asserted-by":"publisher","first-page":"240","DOI":"10.1242\/bio.20147443","article-title":"Coilin association with Box C\/D scaRNA suggests a direct role for the Cajal body marker protein in scaRNP biogenesis","volume":"3","author":"Enwerem","year":"2014","journal-title":"Biol. Open"},{"key":"2021042423185491300_BIO018804C14","doi-asserted-by":"publisher","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":"2021042423185491300_BIO018804C15","doi-asserted-by":"publisher","first-page":"621","DOI":"10.1097\/GIM.0b013e3181ef6079","article-title":"Carrier testing for spinal muscular atrophy","volume":"12","author":"Gitlin","year":"2010","journal-title":"Genet. Med."},{"key":"2021042423185491300_BIO018804C16","doi-asserted-by":"publisher","first-page":"4159","DOI":"10.1091\/mbc.11.12.4159","article-title":"Self-association of coilin reveals a common theme in nuclear body localization","volume":"11","author":"Hebert","year":"2000","journal-title":"Mol. Biol. Cell"},{"key":"2021042423185491300_BIO018804C17","doi-asserted-by":"publisher","first-page":"2720","DOI":"10.1101\/gad.908401","article-title":"Coilin forms the bridge between Cajal bodies and SMN, the spinal muscular atrophy protein","volume":"15","author":"Hebert","year":"2001","journal-title":"Genes Dev."},{"key":"2021042423185491300_BIO018804C18","doi-asserted-by":"publisher","first-page":"329","DOI":"10.1016\/S1534-5807(02)00222-8","article-title":"Coilin methylation regulates nuclear body formation","volume":"3","author":"Hebert","year":"2002","journal-title":"Dev. Cell"},{"key":"2021042423185491300_BIO018804C19","doi-asserted-by":"publisher","first-page":"3221","DOI":"10.1091\/mbc.E06-03-0247","article-title":"Ongoing U snRNP biogenesis is required for the integrity of Cajal bodies","volume":"17","author":"Lemm","year":"2006","journal-title":"Mol. Biol. Cell"},{"key":"2021042423185491300_BIO018804C20","doi-asserted-by":"publisher","first-page":"1269","DOI":"10.1093\/hmg\/7.8.1269","article-title":"The domain encoded by exon 2 of the survival motor neuron protein mediates nucleic acid binding","volume":"7","author":"Lorson","year":"1998","journal-title":"Hum. Mol. Genet."},{"key":"2021042423185491300_BIO018804C21","doi-asserted-by":"publisher","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":"2021042423185491300_BIO018804C22","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1002\/wrna.1139","article-title":"Cajal bodies: where form meets function","volume":"4","author":"Machyna","year":"2013","journal-title":"Wiley Interdiscip. Rev. RNA"},{"key":"2021042423185491300_BIO018804C23","doi-asserted-by":"publisher","first-page":"389","DOI":"10.1016\/j.molcel.2014.10.004","article-title":"The coilin interactome identifies hundreds of small noncoding RNAs that traffic through Cajal bodies","volume":"56","author":"Machyna","year":"2014","journal-title":"Mol. Cell"},{"key":"2021042423185491300_BIO018804C24","doi-asserted-by":"publisher","first-page":"e1000521","DOI":"10.1371\/journal.pbio.1000521","article-title":"WRAP53 is essential for Cajal body formation and for targeting the survival of motor neuron complex to Cajal bodies","volume":"8","author":"Mahmoudi","year":"2010","journal-title":"PLoS Biol."},{"key":"2021042423185491300_BIO018804C25","doi-asserted-by":"publisher","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":"2021042423185491300_BIO018804C26","doi-asserted-by":"publisher","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":"2021042423185491300_BIO018804C27","doi-asserted-by":"publisher","first-page":"919","DOI":"10.1016\/S0140-6736(80)90847-8","article-title":"Classification of spinal muscular atrophies","volume":"315","author":"Pearn","year":"1980","journal-title":"Lancet"},{"key":"2021042423185491300_BIO018804C28","doi-asserted-by":"publisher","first-page":"11167","DOI":"10.1073\/pnas.96.20.11167","article-title":"SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins","volume":"96","author":"Pellizzoni","year":"1999","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"2021042423185491300_BIO018804C29","doi-asserted-by":"publisher","first-page":"1079","DOI":"10.1016\/S0960-9822(01)00316-5","article-title":"The survival of motor neurons (SMN) protein interacts with the snoRNP proteins fibrillarin and GAR1","volume":"11","author":"Pellizzoni","year":"2001","journal-title":"Curr. Biol."},{"key":"2021042423185491300_BIO018804C30","doi-asserted-by":"publisher","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":"2021042423185491300_BIO018804C31","doi-asserted-by":"publisher","first-page":"e1004489","DOI":"10.1371\/journal.pgen.1004489","article-title":"SMA-causing missense mutations in survival motor neuron (Smn) display a wide range of phenotypes when modeled in Drosophila","volume":"10","author":"Praveen","year":"2014","journal-title":"PLoS Genet."},{"key":"2021042423185491300_BIO018804C32","doi-asserted-by":"publisher","first-page":"4351","DOI":"10.1016\/j.febslet.2010.09.034","article-title":"Solution structure of the carboxy-terminal Tudor domain from human Coilin","volume":"584","author":"Shanbhag","year":"2010","journal-title":"FEBS Lett."},{"key":"2021042423185491300_BIO018804C33","doi-asserted-by":"publisher","first-page":"2384","DOI":"10.1128\/MCB.00379-12","article-title":"Telomerase recruitment requires both TCAB1 and Cajal bodies independently","volume":"32","author":"Stern","year":"2012","journal-title":"Mol. Cell. Biol."},{"key":"2021042423185491300_BIO018804C34","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1261\/rna.1354009","article-title":"Dyskeratosis congenita mutations in the H\/ACA domain of human telomerase RNA affect its assembly into a pre-RNP","volume":"15","author":"Trahan","year":"2009","journal-title":"RNA"},{"key":"2021042423185491300_BIO018804C35","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1016\/j.molcel.2009.02.020","article-title":"A conserved WD40 protein binds the Cajal body localization signal of scaRNP particles","volume":"34","author":"Tycowski","year":"2009","journal-title":"Mol. Cell"},{"key":"2021042423185491300_BIO018804C36","doi-asserted-by":"publisher","first-page":"629","DOI":"10.1007\/s00412-012-0387-4","article-title":"Regulated specific proteolysis of the Cajal body marker protein coilin","volume":"121","author":"Velma","year":"2012","journal-title":"Chromosoma"},{"key":"2021042423185491300_BIO018804C37","doi-asserted-by":"publisher","first-page":"644","DOI":"10.1126\/science.1165357","article-title":"A human telomerase holoenzyme protein required for Cajal body localization and telomere synthesis","volume":"323","author":"Venteicher","year":"2009","journal-title":"Science"}],"container-title":["Biology Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1242\/bio.018804","content-type":"unspecified","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/journals.logists.com\/bio\/bio\/article-pdf\/5\/6\/726\/1110813\/bio018804.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/journals.logists.com\/bio\/bio\/article-pdf\/5\/6\/726\/1110813\/bio018804.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,4,24]],"date-time":"2021-04-24T23:18:59Z","timestamp":1619306339000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.biologists.com\/bio\/article\/5\/6\/726\/1280\/SMN-and-coilin-negatively-regulate-dyskerin"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,5,23]]},"references-count":37,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2016,6,15]]}},"URL":"https:\/\/doi.org\/10.1242\/bio.018804","relation":{},"ISSN":["2046-6390"],"issn-type":[{"value":"2046-6390","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,5,23]]}}}