Article
Transcriptional malfunctioning of heat shock protein gene expression in spinocerebellar ataxias.
Cerebellum (London, England) - 1 Jan 2007
Huen N Y Macy, Wong S L Alan, Chan H Y Edwin
Abstract excerpt
Among the various dominantly-inherited spinocerebellar ataxias (SCAs), at least seven of them belong to the polyglutamine disease group and are caused by glutamine-coding CAG triplet repeat expansion. The expanded coding CAG repeat translates into a polyglutamine stretch in the disease protein, which leads to late-onset and progressive neurodegeneration. Expanded polyglutamine adopts a misfolded protein...
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