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Article: Roles of the nucleolus in the CAG RNA-mediated toxicity

TitleRoles of the nucleolus in the CAG RNA-mediated toxicity
Authors
KeywordsNucleolar stress
Nucleolin
P53
Patient fibroblast
Polyglutamine disease
UCE methylation
Issue Date2014
Citation
Biochimica et Biophysica Acta - Molecular Basis of Disease, 2014, v. 1842, n. 6, p. 779-784 How to Cite?
AbstractThe nucleolus is a subnuclear compartment within the cell nucleus that serves as the site for ribosomal RNA (rRNA) transcription and the assembly of ribosome subunits. Apart from its classical role in ribosomal biogenesis, a number of cellular regulatory roles have recently been assigned to the nucleolus, including governing the induction of apoptosis. "Nucleolar stress" is a term that is used to describe a signaling pathway through which the nucleolus communicates with other subcellular compartments, including the mitochondria, to induce apoptosis. It is an effective mechanism for eliminating cells that are incapable of performing protein synthesis efficiently due to ribosome biogenesis defects. The down-regulation of rRNA transcription is a common cause of nucleolar function disruption that subsequently triggers nucleolar stress, and has been associated with the pathogenesis of neurological disorders such as spinocerebellar ataxias (SCAs) and Huntington's diseases (HD). This article discusses recent advances in mechanistic studies of how expanded CAG trinucleotide repeat RNA transcripts trigger nucleolar stress in SCAs, HD and other trinucleotide repeat disorders. This article is part of a Special Issue entitled: Role of the Nucleolus in Human Disease. © 2013 Elsevier B.V.
Persistent Identifierhttp://hdl.handle.net/10722/336123
ISSN
2023 Impact Factor: 4.2
2023 SCImago Journal Rankings: 1.580
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorTsoi, Ho-
dc.contributor.authorChan, Ho Yin Edwin-
dc.date.accessioned2024-01-15T08:23:40Z-
dc.date.available2024-01-15T08:23:40Z-
dc.date.issued2014-
dc.identifier.citationBiochimica et Biophysica Acta - Molecular Basis of Disease, 2014, v. 1842, n. 6, p. 779-784-
dc.identifier.issn0925-4439-
dc.identifier.urihttp://hdl.handle.net/10722/336123-
dc.description.abstractThe nucleolus is a subnuclear compartment within the cell nucleus that serves as the site for ribosomal RNA (rRNA) transcription and the assembly of ribosome subunits. Apart from its classical role in ribosomal biogenesis, a number of cellular regulatory roles have recently been assigned to the nucleolus, including governing the induction of apoptosis. "Nucleolar stress" is a term that is used to describe a signaling pathway through which the nucleolus communicates with other subcellular compartments, including the mitochondria, to induce apoptosis. It is an effective mechanism for eliminating cells that are incapable of performing protein synthesis efficiently due to ribosome biogenesis defects. The down-regulation of rRNA transcription is a common cause of nucleolar function disruption that subsequently triggers nucleolar stress, and has been associated with the pathogenesis of neurological disorders such as spinocerebellar ataxias (SCAs) and Huntington's diseases (HD). This article discusses recent advances in mechanistic studies of how expanded CAG trinucleotide repeat RNA transcripts trigger nucleolar stress in SCAs, HD and other trinucleotide repeat disorders. This article is part of a Special Issue entitled: Role of the Nucleolus in Human Disease. © 2013 Elsevier B.V.-
dc.languageeng-
dc.relation.ispartofBiochimica et Biophysica Acta - Molecular Basis of Disease-
dc.subjectNucleolar stress-
dc.subjectNucleolin-
dc.subjectP53-
dc.subjectPatient fibroblast-
dc.subjectPolyglutamine disease-
dc.subjectUCE methylation-
dc.titleRoles of the nucleolus in the CAG RNA-mediated toxicity-
dc.typeArticle-
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1016/j.bbadis.2013.11.015-
dc.identifier.pmid24269666-
dc.identifier.scopuseid_2-s2.0-84897043536-
dc.identifier.volume1842-
dc.identifier.issue6-
dc.identifier.spage779-
dc.identifier.epage784-
dc.identifier.eissn1879-260X-
dc.identifier.isiWOS:000335431800005-

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