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Article

Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1.

Nature - 10 Apr 2008

Lim Janghoo, Crespo-Barreto Juan, Jafar-Nejad Paymaan, Bowman Aaron B, Richman Ronald, Hill David E, Orr Harry T, Zoghbi Huda Y

Abstract excerpt

Spinocerebellar ataxia type 1 (SCA1) is a dominantly inherited neurodegenerative disease caused by expansion of a glutamine-encoding repeat in ataxin 1 (ATXN1). In all known polyglutamine diseases, the glutamine expansion confers toxic functions onto the protein; however, the mechanism by which this occurs remains enigmatic, in light of the fact that the mutant protein apparently maintains interactions with its...

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Source

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Identifiers

PMID: 18337722
DOI: 10.1038/nature06731
PMCID: PMC2377396

Genes

ATXN1
RBM17

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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