Expression of expanded CAG transcripts triggers nucleolar stress in huntington's disease

Cerebellum

Volumn 12, Issue 3, 2013, Pages 310-312

  Tsoi, Ho ^a   Chan, Ho Yin Edwin ^a  

^a CHINESE UNIVERSITY OF HONG KONG   (Hong Kong)

Author keywords

Huntington's disease; nucleolin; p53; R6 2 transgenic mice; RNA toxicity

Indexed keywords

HUNTINGTIN; NUCLEOLIN; POLYGLUTAMINE; PROTEIN P53; RIBOSOME RNA; TRANSCRIPTION FACTOR; UNCLASSIFIED DRUG; UPSTREAM BINDING FACTOR;

ALZHEIMER DISEASE; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; CAG REPEAT; CELL DEATH; CELL NUCLEUS; CELL STRESS; CONFERENCE PAPER; CONTROLLED STUDY; DEGENERATIVE DISEASE; DOWN REGULATION; GENE EXPRESSION; HUNTINGTON CHOREA; MITOCHONDRION; MOUSE; NONHUMAN; NUCLEOLAR STRESS; PRIORITY JOURNAL; PROMOTER REGION; PROTEIN INTERACTION; RIBOSOMAL RNA TRANSCRIPTION DYSFUNCTION; RNA METHYLATION; RNA STRUCTURE; RNA TRANSCRIPTION; TRANSGENIC MOUSE; UPSTREAM CONTROL ELEMENT;

ANIMALS; CELL NUCLEOLUS; DISEASE MODELS, ANIMAL; HUMANS; HUNTINGTON DISEASE; MICE; MICE, TRANSGENIC; NERVE TISSUE PROTEINS; NUCLEAR PROTEINS; PEPTIDES; RNA, RIBOSOMAL; TRINUCLEOTIDE REPEAT EXPANSION;

EID: 84879068547     PISSN: 14734222     EISSN: 14734230     Source Type: Journal    
DOI: 10.1007/s12311-012-0447-6     Document Type: Conference Paper

References (14)

1. Landles C, Bates GP. Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series. EMBO Rep. 2004;5:958-63.
2. Roos RA. Huntington's disease: a clinical review. Orphanet J Rare Dis. 2010;5:40.
3. Borrell-Pages M, Zala D, Humbert S, Saudou F. Huntington's disease: from huntingtin function and dysfunction to therapeutic strategies. Cell Mol Life Sci. 2006;63:2642-60.
4. Banez-Coronel M, Porta S, Kagerbauer B, Mateu-Huertas E, Pantano L, Ferrer I, et al. A pathogenic mechanism in Huntington's disease involves small CAG-repeated RNAs with neurotoxic activity. PLoS Genet. 2012;8:e1002481.
5. Li LB, Yu Z, Teng X, Bonini NM. RNA toxicity is a component of ataxin-3 degeneration in Drosophila. Nature. 2008;453:1107-11.
6. Zhang Y, Lu H. Signaling to p53: ribosomal proteins find their way. Cancer Cell. 2009;16:369-77.
7. Speidel D. Transcription-independent p53 apoptosis: an alternative route to death. Trends Cell Biol. 2010;20:14-24.
8. Ruggero D, Grisendi S, Piazza F, Rego E, Mari F, Rao PH, et al. Dyskeratosis congenita and cancer in mice deficient in ribosomal RNA modification. Science. 2003;299:259-62.
9. Parlato R, Kreiner G, Erdmann G, Rieker C, Stotz S, Savenkova E, et al. Activation of an endogenous suicide response after perturbation of rRNA synthesis leads to neurodegeneration in mice. J Neurosci. 2008;28:12759-64.
10. Pietrzak M, Rempala G, Nelson PT, Zheng JJ, Hetman M. Epigenetic silencing of nucleolar rRNA genes in Alzheimer's disease. PLoS One. 2011;6:e22585.
11. Tsoi H, Lau TC, Tsang SY, Lau KF, Chan HY. CAG expansion induces nucleolar stress in polyglutamine diseases. Proc Natl Acad Sci U S A. 2012;109:13428-33.
12. Ruggero D, Pandolfi PP. Does the ribosome translate cancer? Nat Rev Cancer. 2003;3:179-92.
13. Santoro R, Grummt I. Epigenetic mechanism of rRNA gene silencing: temporal order of NoRC-mediated histone modification, chromatin remodeling, and DNA methylation. Mol Cell Biol. 2005;25:2539-46.
14. Lee J, Hwang YJ, Boo JH, Han D, Kwon OK, Todorova K, et al. Dysregulation of upstream binding factor-1 acetylation at K352 is linked to impaired ribosomal DNA transcription in Huntington's disease. Cell Death Differ. 2011;18:1726-35.