{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T16:38:11Z","timestamp":1790440691050,"version":"4.1.0"},"reference-count":113,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2017,12,1]],"date-time":"2017-12-01T00:00:00Z","timestamp":1512086400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/501100006527","name":"Direcci\u00f3n General de Investigaci\u00f3n","doi-asserted-by":"publisher","award":["BFU2014-54754-P"],"award-info":[{"award-number":["BFU2014-54754-P"]}],"id":[{"id":"10.13039\/501100006527","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Centro de Investigaci\u00f3n Biom\u00e9dica en Red sobre Enfermedades Neurodegenerativas","award":["CB06\/05\/0037"],"award-info":[{"award-number":["CB06\/05\/0037"]}]},{"DOI":"10.13039\/501100003329","name":"Ministerio de Econom\u00eda y Competitividad","doi-asserted-by":"publisher","award":["SAF2015-70801-R"],"award-info":[{"award-number":["SAF2015-70801-R"]}],"id":[{"id":"10.13039\/501100003329","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.jp","clinicalkey.com","clinicalkey.es","clinicalkey.com.au","clinicalkey.fr","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurobiology of Disease"],"published-print":{"date-parts":[[2017,12]]},"DOI":"10.1016\/j.nbd.2017.08.004","type":"journal-article","created":{"date-parts":[[2017,8,18]],"date-time":"2017-08-18T10:19:33Z","timestamp":1503051573000},"page":"83-99","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":31,"special_numbering":"C","title":["Cellular bases of the RNA metabolism dysfunction in motor neurons of a murine model of spinal muscular atrophy: Role of Cajal bodies and the nucleolus"],"prefix":"10.1016","volume":"108","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0802-7975","authenticated-orcid":false,"given":"Olga","family":"Tapia","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Josep Oriol","family":"Narc\u00eds","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Javier","family":"Riancho","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olga","family":"Tarabal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L\u00eddia","family":"Piedrafita","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jordi","family":"Calder\u00f3","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria T.","family":"Berciano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel","family":"Lafarga","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2017.08.004_bb0005","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1111\/j.1600-0854.2010.01063.x","article-title":"Nucleolar disruption ensures nuclear accumulation of p21 upon DNA damage","volume":"11","author":"Abella","year":"2010","journal-title":"Traffic"},{"key":"10.1016\/j.nbd.2017.08.004_bb0010","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/j.molcel.2011.12.012","article-title":"Immobilization of proteins in the nucleolus by ribosomal intergenic spacer noncoding RNA","volume":"45","author":"Audas","year":"2012","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2017.08.004_bb0015","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1111\/j.1750-3639.2010.00461.x","article-title":"Nucleolar disruption and Cajal body disassembly are nuclear hallmarks of DNA damage-induced neurodegeneration in purkinje cells","volume":"21","author":"Baltanas","year":"2011","journal-title":"Brain Pathol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0020","doi-asserted-by":"crossref","first-page":"1000773","DOI":"10.1371\/journal.pgen.1000773","article-title":"Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy","volume":"5","author":"B\u00e4umer","year":"2009","journal-title":"PLoS Genet."},{"key":"10.1016\/j.nbd.2017.08.004_bb0025","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1016\/j.jsb.2006.12.008","article-title":"Cajal body number and nucleolar size correlate with the cell body mass in human sensory ganglia neurons","volume":"158","author":"Berciano","year":"2007","journal-title":"J. Struct. Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb9000","doi-asserted-by":"crossref","first-page":"817","DOI":"10.1083\/jcb.131.4.817","article-title":"Mutational analysis of p80 coilin indicates a functional interaction between coiled bodies and the nucleolus","volume":"131","author":"Bohmann","year":"1995","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0030","doi-asserted-by":"crossref","first-page":"957","DOI":"10.1083\/jcb.200207028","article-title":"Symmetrical dimethylarginine methylation is required for the localization of SMN in Cajal bodies and pre-mRNA splicing","volume":"159","author":"Boisvert","year":"2002","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0035","doi-asserted-by":"crossref","first-page":"574","DOI":"10.1038\/nrm2184","article-title":"The multifunctional nucleolus","volume":"8","author":"Boisvert","year":"2007","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0040","doi-asserted-by":"crossref","first-page":"641","DOI":"10.1093\/hmg\/ddq508","article-title":"Impaired minor tri-snRNP assembly generates differential splicing defects of U12-type introns in lymphoblasts derived from a type I SMA patient","volume":"20","author":"Boulisfane","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2017.08.004_bb0045","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1016\/j.molcel.2004.11.013","article-title":"PHAX and CRM1 are required sequentially to transport U3 snoRNA to nucleoli","volume":"16","author":"Boulon","year":"2004","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2017.08.004_bb0050","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1016\/j.molcel.2010.09.024","article-title":"The nucleolus under stress","volume":"40","author":"Boulon","year":"2010","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2017.08.004_bb0055","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0178925","article-title":"SUMO regulates p21Cip1 intracellular distribution and with p21Cip1 facilitates multiprotein complex formation in the nucleolus upon DNA damage","volume":"12","author":"Brun","year":"2017","journal-title":"PlosOne"},{"key":"10.1016\/j.nbd.2017.08.004_bb0060","doi-asserted-by":"crossref","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":"10.1016\/j.nbd.2017.08.004_bb0065","doi-asserted-by":"crossref","first-page":"\u00e7\u00e7817","DOI":"10.1016\/j.bbadis.2013.08.014","article-title":"Activation of the tumor suppressor p53 upon impairment of ribosome biogenesis","volume":"1842","author":"Bursac","year":"2014","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.nbd.2017.08.004_bb0070","doi-asserted-by":"crossref","first-page":"69536","DOI":"10.18632\/oncotarget.11766","article-title":"MXD1 localizes in the nucleolus, binds UBF and impairs rRNA synthesis","volume":"7","author":"Carmen Lafita-Navarro","year":"2016","journal-title":"Oncotarget"},{"key":"10.1016\/j.nbd.2017.08.004_bb0075","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1083\/jcb.117.1.1","article-title":"Transcription-dependent colocalization of the U1, U2, U4\/U6, and U5 snRNPs in coiled bodies","volume":"117","author":"Carmo-Fonseca","year":"1992","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0080","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1083\/jcb.147.4.715","article-title":"The spinal muscular atrophy disease gene product, SMN: a link between snRNP biogenesis and the Cajal (coiled) body","volume":"147","author":"Carvalho","year":"1999","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0085","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1016\/j.neuroscience.2006.02.030","article-title":"Nuclear organization and dynamics of transcription sites in rat sensory ganglia neurons detected by incorporation of 5\u2032-fluorouridine into nascent RNA","volume":"140","author":"Casafont","year":"2006","journal-title":"Neuroscience"},{"key":"10.1016\/j.nbd.2017.08.004_bb0090","doi-asserted-by":"crossref","first-page":"574","DOI":"10.1111\/cns.12242","article-title":"Gem depletion: amyotrophic lateral sclerosis and spinal muscular atrophy crossover","volume":"20","author":"Cauchi","year":"2014","journal-title":"CNS. Neurosci. Ther."},{"key":"10.1016\/j.nbd.2017.08.004_bb0095","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1007\/s13311-015-0399-x","article-title":"Chronic treatment with the AMPK agonist AICAR prevents skeletal muscle pathology but fails to improve clinical outcome in a mouse model of severe spinal muscular atrophy","volume":"13","author":"Cervero","year":"2016","journal-title":"Neurotherapeutics"},{"key":"10.1016\/j.nbd.2017.08.004_bb0100","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1101\/gad.1884910","article-title":"A degron created by SMN2 exon 7 skipping is a principal contributor to spinal muscular atrophy severity","volume":"24","author":"Cho","year":"2010","journal-title":"Genes Dev."},{"key":"10.1016\/j.nbd.2017.08.004_bb0105","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1083\/jcb.200604099","article-title":"UV-induced fragmentation of Cajal bodies","volume":"175","author":"Cioce","year":"2006","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0110","doi-asserted-by":"crossref","first-page":"1585","DOI":"10.1111\/j.1600-0854.2009.00972.x","article-title":"The SMN protein is a key regulator of nuclear architecture in differentiating neuroblastoma cells","volume":"10","author":"Clelland","year":"2009","journal-title":"Traffic"},{"key":"10.1016\/j.nbd.2017.08.004_bb0115","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1007\/s004180050437","article-title":"Ultrastructural analysis of nucleolar transcription in cells microinjected with 5-bromo-UTP","volume":"113","author":"Cmarko","year":"2000","journal-title":"Histochem. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0120","doi-asserted-by":"crossref","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":"10.1016\/j.nbd.2017.08.004_bb0125","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.nbd.2017.08.004_bb0130","doi-asserted-by":"crossref","first-page":"6014","DOI":"10.1128\/MCB.22.16.6014-6022.2002","article-title":"Stress-dependent nucleolin mobilization mediated by p53-nucleolin complex formation","volume":"22","author":"Daniely","year":"2002","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0135","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1007\/s00223-011-9522-0","article-title":"Osteocyte deficiency in hip fractures","volume":"89","author":"Delgado-Calle","year":"2011","journal-title":"Calcif. Tissue Int."},{"key":"10.1016\/j.nbd.2017.08.004_bb0140","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1093\/nar\/gkw731","article-title":"RNA-sequencing of a mouse-model of spinal muscular atrophy reveals tissue-wide changes in splicing of U12-dependent introns","volume":"45","author":"Doktor","year":"2017","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.nbd.2017.08.004_bb0145","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1016\/j.molcel.2008.10.017","article-title":"A human snoRNA with microRNA-like functions","volume":"32","author":"Ender","year":"2008","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2017.08.004_bb0150","doi-asserted-by":"crossref","first-page":"1686","DOI":"10.1016\/j.cell.2016.04.047","article-title":"Coexisting liquid phases underlie nucleolar subcompartments","volume":"165","author":"Feric","year":"2016","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2017.08.004_bb0155","doi-asserted-by":"crossref","first-page":"718","DOI":"10.1002\/wrna.87","article-title":"Biogenesis of spliceosomal small nuclear ribonucleoproteins","volume":"2","author":"Fischer","year":"2011","journal-title":"Wiley Interdiscip. Rev. RNA"},{"key":"10.1016\/j.nbd.2017.08.004_bb0160","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1016\/S0959-437X(02)00301-5","article-title":"The molecular bases of spinal muscular atrophy","volume":"12","author":"Frugier","year":"2002","journal-title":"Curr. Opin. Genet. Dev."},{"key":"10.1016\/j.nbd.2017.08.004_bb0165","doi-asserted-by":"crossref","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"},{"key":"10.1016\/j.nbd.2017.08.004_bb0170","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1146\/annurev.cellbio.16.1.273","article-title":"Cajal bodies: the first 100 years","volume":"16","author":"Gall","year":"2000","journal-title":"Annu. Rev. Cell Dev. Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0175","doi-asserted-by":"crossref","first-page":"975","DOI":"10.1083\/jcb.137.5.975","article-title":"Targeting of U2AF65 to sites of active splicing in the nucleus","volume":"137","author":"Gama-Carvalho","year":"1997","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0180","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1186\/s40478-015-0257-4","article-title":"Altered machinery of protein synthesis is region- and stage-dependent and is associated with alpha-synuclein oligomers in Parkinson's disease","volume":"3","author":"Garcia-Esparcia","year":"2015","journal-title":"Acta Neuropathol. Commun."},{"key":"10.1016\/j.nbd.2017.08.004_bb0185","doi-asserted-by":"crossref","first-page":"1632","DOI":"10.1261\/rna.043398.113","article-title":"A cluster of methylations in the domain IV of 25S rRNA is required for ribosome stability","volume":"20","author":"Gigova","year":"2014","journal-title":"RNA"},{"key":"10.1016\/j.nbd.2017.08.004_bb0190","doi-asserted-by":"crossref","first-page":"1070","DOI":"10.1091\/mbc.e10-08-0731","article-title":"Coilin participates in the suppression of RNA polymerase I in response to cisplatin-induced DNA damage","volume":"22","author":"Gilder","year":"2011","journal-title":"Mol. Biol. Cell"},{"key":"10.1016\/j.nbd.2017.08.004_bb0195","doi-asserted-by":"crossref","first-page":"1160","DOI":"10.2174\/15665240113139990044","article-title":"At the \"junction\" of spinal muscular atrophy pathogenesis: the role of neuromuscular junction dysfunction in SMA disease progression","volume":"13","author":"Goulet","year":"2013","journal-title":"Curr. Mol. Med."},{"key":"10.1016\/j.nbd.2017.08.004_bb0200","doi-asserted-by":"crossref","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":"10.1016\/j.nbd.2017.08.004_bb0205","article-title":"Toward an understanding of regulating Cajal body activity by protein modification","volume":"1\u201318","author":"Hebert","year":"2017","journal-title":"RNA Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0210","doi-asserted-by":"crossref","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":"10.1016\/j.nbd.2017.08.004_bb0215","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1111\/bpa.12335","article-title":"Altered machinery of protein synthesis in Alzheimer's: from the nucleolus to the ribosome","volume":"26","author":"Hernandez-Ortega","year":"2016","journal-title":"Brain Pathol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0220","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1016\/j.tins.2012.01.002","article-title":"Emerging roles of the neuronal nucleolus","volume":"35","author":"Hetman","year":"2012","journal-title":"Trends Neurosci."},{"key":"10.1016\/j.nbd.2017.08.004_bb0225","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1007\/s00412-011-0327-8","article-title":"An intranucleolar body associated with rDNA","volume":"120","author":"Hutten","year":"2011","journal-title":"Chromosoma"},{"key":"10.1016\/j.nbd.2017.08.004_bb0230","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1083\/jcb.142.2.319","article-title":"Nopp140 functions as a molecular link between the nucleolus and the coiled bodies","volume":"142","author":"Isaac","year":"1998","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0235","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/j.braindev.2010.06.009","article-title":"New insights into the pathogenesis of spinal muscular atrophy","volume":"33","author":"Ito","year":"2011","journal-title":"Brain and Development"},{"key":"10.1016\/j.nbd.2017.08.004_bb0240","doi-asserted-by":"crossref","first-page":"E2347","DOI":"10.1073\/pnas.1613181114","article-title":"SMN deficiency in severe models of spinal muscular atrophy causes widespread intron retention and DNA damage","volume":"114","author":"Jangi","year":"2017","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2017.08.004_bb9100","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1083\/jcb.113.4.715","article-title":"Evolutionary conservation of the human nucleolar protein fibrillarin and its functional expression in yeast","volume":"113","author":"Jansen","year":"1991","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0245","doi-asserted-by":"crossref","first-page":"4906","DOI":"10.1093\/hmg\/ddq425","article-title":"A feedback loop regulates splicing of the spinal muscular atrophy-modifying gene, SMN2","volume":"19","author":"Jodelka","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2017.08.004_bb0250","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1242\/jcs.52.1.373","article-title":"The quantitative relationship of the fibrillar centres and other nucleolar components to changes in growth conditions, serum deprivation and low doses of actinomycin D in cultured diploid human fibroblasts (strain MRC-5)","volume":"52","author":"Jordan","year":"1981","journal-title":"J. Cell Sci."},{"key":"10.1016\/j.nbd.2017.08.004_bb0255","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1038\/nn1867","article-title":"Activity-dependent AIDA-1 nuclear signaling regulates nucleolar numbers and protein synthesis in neurons","volume":"10","author":"Jordan","year":"2007","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.nbd.2017.08.004_bb0260","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1016\/S0092-8674(02)00718-3","article-title":"Small nucleolar RNAs: an abundant group of noncoding RNAs with diverse cellular functions","volume":"109","author":"Kiss","year":"2002","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2017.08.004_bb0265","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1002\/cne.903080302","article-title":"Organization of nucleoli and nuclear bodies in osmotically stimulated supraoptic neurons of the rat","volume":"308","author":"Lafarga","year":"1991","journal-title":"J. Comp. Neurol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0270","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1007\/s004410050849","article-title":"Apoptosis induced by methylazoxymethanol in developing rat cerebellum: organization of the cell nucleus and its relationship to DNA and rRNA degradation","volume":"289","author":"Lafarga","year":"1997","journal-title":"Cell Tissue Res."},{"key":"10.1016\/j.nbd.2017.08.004_bb0275","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1023\/A:1006937032068","article-title":"Acute osmotic\/stress stimuli induce a transient decrease of transcriptional activity in the neurosecretory neurons of supraoptic nuclei","volume":"27","author":"Lafarga","year":"1998","journal-title":"J. Neurocytol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0280","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1007\/s00412-009-0212-x","article-title":"Cajal's contribution to the knowledge of the neuronal cell nucleus","volume":"118","author":"Lafarga","year":"2009","journal-title":"Chromosoma"},{"key":"10.1016\/j.nbd.2017.08.004_bb0285","first-page":"1","article-title":"Cajal bodies in neurons","author":"Lafarga","year":"2017","journal-title":"RNA Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0290","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1093\/hmg\/ddi078","article-title":"SMNDelta7, the major product of the centromeric survival motor neuron (SMN2) gene, extends survival in mice with spinal muscular atrophy and associates with full-length SMN","volume":"14","author":"Le","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2017.08.004_bb0295","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/0092-8674(95)90460-3","article-title":"Identification and characterization of spinal muscular atrophy-determining gene","volume":"80","author":"Lefebvre","year":"1995","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2017.08.004_bb0300","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.nbd.2017.08.004_bb9200","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1006\/meth.2001.1262","article-title":"Analysis of Relative Gene Expression Data Using Real-Time Quantitative PCR and the 2\u2212\u0394\u0394CT Method","volume":"25","author":"Livak","year":"2001","journal-title":"Methods"},{"key":"10.1016\/j.nbd.2017.08.004_bb0305","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":"EMBO J."},{"key":"10.1016\/j.nbd.2017.08.004_bb0310","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1016\/j.cell.2012.09.012","article-title":"An SMN-dependent U12 splicing event essential for motor circuit function","volume":"151","author":"Lotti","year":"2012","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2017.08.004_bb0315","doi-asserted-by":"crossref","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":"10.1016\/j.nbd.2017.08.004_bb0320","doi-asserted-by":"crossref","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":"10.1016\/j.nbd.2017.08.004_bb0325","first-page":"1","article-title":"Assembly and trafficking of box C\/D and H\/ACA snoRNPs","author":"Massenet","year":"2017","journal-title":"RNA Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0330","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1038\/nrm3742","article-title":"A day in the life of the spliceosome","volume":"15","author":"Matera","year":"2014","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0335","doi-asserted-by":"crossref","first-page":"933","DOI":"10.1101\/gad.333205","article-title":"The nucleolus as a stress sensor: JNK2 inactivates the transcription factor TIF-IA and down-regulates rRNA synthesis","volume":"19","author":"Mayer","year":"2005","journal-title":"Genes Dev."},{"key":"10.1016\/j.nbd.2017.08.004_bb0340","doi-asserted-by":"crossref","first-page":"1598","DOI":"10.1101\/gad.283838.116","article-title":"Nucleolar organizer regions: genomic 'dark matter' requiring illumination","volume":"30","author":"McStay","year":"2016","journal-title":"Genes Dev."},{"key":"10.1016\/j.nbd.2017.08.004_bb0345","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.nbd.2017.08.004_bb0350","doi-asserted-by":"crossref","first-page":"5120","DOI":"10.1093\/emboj\/18.18.5120","article-title":"Nucleolar localization signals of box H\/ACA small nucleolar RNAs","volume":"18","author":"Narayanan","year":"1999","journal-title":"EMBO J."},{"key":"10.1016\/j.nbd.2017.08.004_bb0355","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1007\/s00412-004-0285-5","article-title":"Targeting SMN to Cajal bodies and nuclear gems during neuritogenesis","volume":"112","author":"Navascues","year":"2004","journal-title":"Chromosoma"},{"key":"10.1016\/j.nbd.2017.08.004_bb0360","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/j.jsb.2008.04.013","article-title":"SUMO-1 transiently localizes to Cajal bodies in mammalian neurons","volume":"163","author":"Navascues","year":"2008","journal-title":"J. Struct. Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0365","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1016\/j.stem.2015.08.003","article-title":"Genome-wide RNA-seq of human motor neurons implicates selective ER stress activation in spinal muscular atrophy","volume":"17","author":"Ng","year":"2015","journal-title":"Cell Stem Cell"},{"issue":"11390","key":"10.1016\/j.nbd.2017.08.004_bb0370","article-title":"Involvement of human ribosomal proteins in nucleolar structure and p53-dependent nucleolar stress","volume":"7","author":"Nicolas","year":"2016","journal-title":"Nat. Commun."},{"key":"10.1016\/j.nbd.2017.08.004_bb0375","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1091\/mbc.e10-07-0560","article-title":"In vivo kinetics of U4\/U6.U5 tri-snRNP formation in Cajal bodies","volume":"22","author":"Novotny","year":"2011","journal-title":"Mol. Biol. Cell"},{"key":"10.1016\/j.nbd.2017.08.004_bb0380","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1039\/C6MB00740F","article-title":"Crosstalk between the nucleolus and the DNA damage response","volume":"13","author":"Ogawa","year":"2017","journal-title":"Mol. BioSyst."},{"key":"10.1016\/j.nbd.2017.08.004_bb0385","doi-asserted-by":"crossref","DOI":"10.1126\/stke.2242004pe10","article-title":"Sensing cellular stress: another new function for the nucleolus?","volume":"2004","author":"Olson","year":"2004","journal-title":"Sci. STKE"},{"key":"10.1016\/j.nbd.2017.08.004_bb0390","doi-asserted-by":"crossref","first-page":"3879","DOI":"10.1093\/nar\/gkq1355","article-title":"Identification of human miRNA precursors that resemble box C\/D snoRNAs","volume":"39","author":"Ono","year":"2011","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.nbd.2017.08.004_bb0395","doi-asserted-by":"crossref","first-page":"1961","DOI":"10.1007\/s00018-013-1474-2","article-title":"Proteasome inhibition induces DNA damage and reorganizes nuclear architecture and protein synthesis machinery in sensory ganglion neurons","volume":"71","author":"Palanca","year":"2014","journal-title":"Cell. Mol. Life Sci."},{"key":"10.1016\/j.nbd.2017.08.004_bb0400","doi-asserted-by":"crossref","first-page":"848","DOI":"10.1016\/j.bbadis.2013.11.016","article-title":"Reactive nucleolar and Cajal body responses to proteasome inhibition in sensory ganglion neurons","volume":"1842","author":"Palanca","year":"2014","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.nbd.2017.08.004_bb0405","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1007\/s00109-012-0981-1","article-title":"Nucleolar activity in neurodegenerative diseases: a missing piece of the puzzle?","volume":"91","author":"Parlato","year":"2013","journal-title":"J. Mol. Med. (Berl)"},{"key":"10.1016\/j.nbd.2017.08.004_bb0410","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1016\/j.bbadis.2013.12.014","article-title":"How Parkinson's disease meets nucleolar stress","volume":"1842","author":"Parlato","year":"2014","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.nbd.2017.08.004_bb0415","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1002\/1096-9861(20010205)430:2<250::AID-CNE1029>3.0.CO;2-L","article-title":"Neuronal body size correlates with the number of nucleoli and Cajal bodies, and with the organization of the splicing machinery in rat trigeminal ganglion neurons","volume":"430","author":"Pena","year":"2001","journal-title":"J. Comp. Neurol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0420","doi-asserted-by":"crossref","first-page":"1561","DOI":"10.1083\/jcb.151.7.1561","article-title":"In vivo analysis of Cajal body movement, separation, and joining in live human cells","volume":"151","author":"Platani","year":"2000","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0425","doi-asserted-by":"crossref","first-page":"831","DOI":"10.1083\/jcb.200610053","article-title":"A Drosophila melanogaster model of spinal muscular atrophy reveals a function for SMN in striated muscle","volume":"176","author":"Rajendra","year":"2007","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0430","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1016\/1047-8477(90)90066-L","article-title":"Association between the nucleolus and the coiled body","volume":"104","author":"Raska","year":"1990","journal-title":"J. Struct. Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0435","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1016\/S0074-7696(06)55004-1","article-title":"New insights into nucleolar architecture and activity","volume":"255","author":"Raska","year":"2006","journal-title":"Int. Rev. Cytol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0440","doi-asserted-by":"crossref","first-page":"1181","DOI":"10.1093\/hmg\/ddp009","article-title":"The loss of the snoRNP chaperone Nopp140 from Cajal bodies of patient fibroblasts correlates with the severity of spinal muscular atrophy","volume":"18","author":"Renvoise","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2017.08.004_bb0445","doi-asserted-by":"crossref","DOI":"10.3389\/fncel.2014.00346","article-title":"Compensatory motor neuron response to chromatolysis in the murine hSOD1 G93A model of amyotrophic lateral sclerosis","volume":"8","author":"Riancho","year":"2014","journal-title":"Front. Cell. Neurosci."},{"key":"10.1016\/j.nbd.2017.08.004_bb0450","doi-asserted-by":"crossref","first-page":"250","DOI":"10.3389\/fncel.2015.00250","article-title":"Neuroprotective effect of Bexarotene in the SOD1(G93A) mouse model of amyotrophic lateral sclerosis","volume":"9","author":"Riancho","year":"2015","journal-title":"Front. Cell. Neurosci."},{"key":"10.1016\/j.nbd.2017.08.004_bb0455","article-title":"Why do motor neurons degenerate? Actualization in the pathogenesis of amyotrophic lateral sclerosis","author":"Riancho","year":"2016","journal-title":"Neurologia"},{"key":"10.1016\/j.nbd.2017.08.004_bb0460","doi-asserted-by":"crossref","first-page":"1484","DOI":"10.1016\/j.celrep.2017.01.035","article-title":"Single-cell analysis of SMN reveals its broader role in neuromuscular disease","volume":"18","author":"Rodriguez-Muela","year":"2017","journal-title":"Cell Rep."},{"key":"10.1016\/j.nbd.2017.08.004_bb0465","doi-asserted-by":"crossref","first-page":"1197","DOI":"10.1002\/bies.201600144","article-title":"Cajal body function in genome organization and transcriptome diversity","volume":"38","author":"Sawyer","year":"2016","journal-title":"BioEssays"},{"key":"10.1016\/j.nbd.2017.08.004_bb0470","doi-asserted-by":"crossref","first-page":"2395","DOI":"10.1091\/mbc.e04-11-0992","article-title":"Dynamic sorting of nuclear components into distinct nucleolar caps during transcriptional inhibition","volume":"16","author":"Shav-Tal","year":"2005","journal-title":"Mol. Biol. Cell"},{"key":"10.1016\/j.nbd.2017.08.004_bb0475","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.nbd.2017.08.004_bb0480","doi-asserted-by":"crossref","first-page":"1540","DOI":"10.1242\/jcs.001529","article-title":"A regulatory role for CRM1 in the multi-directional trafficking of splicing snRNPs in the mammalian nucleus","volume":"120","author":"Sleeman","year":"2007","journal-title":"J. Cell Sci."},{"key":"10.1016\/j.nbd.2017.08.004_bb0485","doi-asserted-by":"crossref","first-page":"1065","DOI":"10.1016\/S0960-9822(99)80475-8","article-title":"Newly assembled snRNPs associate with coiled bodies before speckles, suggesting a nuclear snRNP maturation pathway","volume":"9","author":"Sleeman","year":"1999","journal-title":"Curr. Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0490","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1007\/s00418-016-1407-x","article-title":"Nucleolar DNA: the host and the guests","volume":"145","author":"Smirnov","year":"2016","journal-title":"Histochem. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0495","doi-asserted-by":"crossref","first-page":"1624","DOI":"10.1093\/brain\/awf155","article-title":"Neuronal death is enhanced and begins during foetal development in type I spinal muscular atrophy spinal cord","volume":"125","author":"Soler-Botija","year":"2002","journal-title":"Brain"},{"key":"10.1016\/j.nbd.2017.08.004_bb0500","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1080\/19491034.2015.1082695","article-title":"Comparative ultrastructure of CRM1-nucleolar bodies (CNoBs), Intranucleolar bodies (INBs) and hybrid PML\/p62 bodies uncovers new facets of nuclear body dynamic and diversity","volume":"6","author":"Souquere","year":"2015","journal-title":"Nucleus"},{"key":"10.1016\/j.nbd.2017.08.004_bb0505","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1007\/s00412-010-0276-7","article-title":"Nucleolar targeting of coilin is regulated by its hypomethylation state","volume":"119","author":"Tapia","year":"2010","journal-title":"Chromosoma"},{"key":"10.1016\/j.nbd.2017.08.004_bb0510","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1007\/s00418-012-0921-8","article-title":"Reorganization of Cajal bodies and nucleolar targeting of coilin in motor neurons of type I spinal muscular atrophy","volume":"137","author":"Tapia","year":"2012","journal-title":"Histochem. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0515","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1097\/NEN.0000000000000074","article-title":"Mechanisms involved in spinal cord central synapse loss in a mouse model of spinal muscular atrophy","volume":"73","author":"Tarabal","year":"2014","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0520","doi-asserted-by":"crossref","first-page":"8691","DOI":"10.1523\/JNEUROSCI.0417-15.2015","article-title":"Disease mechanisms and therapeutic approaches in spinal muscular atrophy","volume":"35","author":"Tisdale","year":"2015","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2017.08.004_bb0525","first-page":"1","article-title":"The Cajal body and the nucleolus: \"In a relationship\" or \"It's complicated\"?","author":"Trinkle-Mulcahy","year":"2017","journal-title":"RNA Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0530","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1002\/emmm.201202303","article-title":"Spliceosome integrity is defective in the motor neuron diseases ALS and SMA","volume":"5","author":"Tsuiji","year":"2013","journal-title":"EMBO Mol. Med."},{"key":"10.1016\/j.nbd.2017.08.004_bb0535","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1006\/excr.2001.5186","article-title":"Nuclear gems and Cajal (coiled) bodies in fetal tissues: nucleolar distribution of the spinal muscular atrophy protein, SMN","volume":"265","author":"Young","year":"2001","journal-title":"Exp. Cell Res."},{"key":"10.1016\/j.nbd.2017.08.004_bb0540","doi-asserted-by":"crossref","first-page":"1279","DOI":"10.1083\/jcb.152.6.1279","article-title":"Internal modification of U2 small nuclear (sn)RNA occurs in nucleoli of Xenopus oocytes","volume":"152","author":"Yu","year":"2001","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2017.08.004_bb0545","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.nbd.2017.08.004_bb0550","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1016\/j.pneurobio.2016.05.004","article-title":"ALS: a bucket of genes, environment, metabolism and unknown ingredients","volume":"142","author":"Zufiria","year":"2016","journal-title":"Prog. Neurobiol."}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996117301870?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996117301870?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2022,8,1]],"date-time":"2022-08-01T11:13:07Z","timestamp":1659352387000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0969996117301870"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,12]]},"references-count":113,"alternative-id":["S0969996117301870"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2017.08.004","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2017,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Cellular bases of the RNA metabolism dysfunction in motor neurons of a murine model of spinal muscular atrophy: Role of Cajal bodies and the nucleolus","name":"articletitle","label":"Article Title"},{"value":"Neurobiology of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nbd.2017.08.004","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2017 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}