Cell. 2006 Dec 29;127(7):1299-300.

Hosting neurotoxicity in polyglutamine disease.

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Department of Biology, University of Pennsylvania, Philadelphia, PA 19104, USA.

Abstract

Polyglutamine diseases are caused by an expanded glutamine domain thought to confer a toxic activity onto the respective disease proteins. In this issue, propose that toxicity of the polyglutamine protein Ataxin-1 may not be due to abberant protein interactions mediated by the polyglutamine expansion. Instead, they suggest that toxicity is solely due to interactions of Ataxin-1 with its normal binding partners.

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Cell. 2006 Dec 29;127(7):1335-47.

PMID:: 17190592; [PubMed - indexed for MEDLINE]

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ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology. [Cell. 2006]
ATAXIN-1 interacts with the repressor Capicua in its native complex to cause SCA1 neuropathology.
Lam YC, Bowman AB, Jafar-Nejad P, Lim J, Richman R, Fryer JD, Hyun ED, Duvick LA, Orr HT, Botas J, et al. Cell. 2006 Dec 29; 127(7):1335-47.
Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1. [Nature. 2008]
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Lim J, Crespo-Barreto J, Jafar-Nejad P, Bowman AB, Richman R, Hill DE, Orr HT, Zoghbi HY. Nature. 2008 Apr 10; 452(7188):713-8. Epub 2008 Mar 12.
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The spinocerebellar ataxia type 1 protein, ataxin-1, has RNA-binding activity that is inversely affected by the length of its polyglutamine tract.
Yue S, Serra HG, Zoghbi HY, Orr HT. Hum Mol Genet. 2001 Jan 1; 10(1):25-30.
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Cited by 1 PubMed Central article

Phosphorylation of S776 and 14-3-3 binding modulate ataxin-1 interaction with splicing factors. [PLoS One. 2009]
Phosphorylation of S776 and 14-3-3 binding modulate ataxin-1 interaction with splicing factors.
de Chiara C, Menon RP, Strom M, Gibson TJ, Pastore A. PLoS One. 2009 Dec 23; 4(12):e8372. Epub 2009 Dec 23.

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Hosting neurotoxicity in polyglutamine disease.

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