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Article

Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice.

PloS one - 1 Jan 2013

Nóbrega Clévio, Nascimento-Ferreira Isabel, Onofre Isabel, Albuquerque David, Hirai Hirokazu, Déglon Nicole, de Almeida Luís Pereira

Abstract excerpt

Machado-Joseph disease (MJD) or spinocerebellar ataxia type 3 (SCA3) is an autosomal dominantly-inherited neurodegenerative disorder caused by the over-repetition of a CAG codon in the MJD1 gene. This expansion translates into a polyglutamine tract that confers a toxic gain-of-function to the mutant protein--ataxin-3, leading to neurodegeneration in specific brain regions, with particular severity in the...

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Identifiers

PMID: 23349684
DOI: 10.1371/journal.pone.0052396
PMCID: PMC3551966

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Source receipt: pubmed, retrieved 7 Sept 2026.

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Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice.PMID 23349684Read ↗

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