کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
1904763 1534659 2014 6 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Roles of the nucleolus in the CAG RNA-mediated toxicity
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی سالمندی
پیش نمایش صفحه اول مقاله
Roles of the nucleolus in the CAG RNA-mediated toxicity
چکیده انگلیسی


• Polyglutamine toxicity is caused by the expression of both mutant RNA and protein.
• Expanded CAG RNAs form foci in the nucleolus.
• Expanded CAG RNAs trigger nucleolar stress by competing with UCE for nucleolin.
• Expanded CAG RNAs cause hypermethylation of UCE of rRNA promoter.
• Expanded CAG RNAs down-regulate rRNA transcription.

The 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.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1842, Issue 6, June 2014, Pages 779–784
نویسندگان
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