The role of chaperones in polyglutamine disease

P Opal, HY Zoghbi - Trends in molecular medicine, 2002 - cell.com
P Opal, HY Zoghbi
Trends in molecular medicine, 2002cell.com
The 'triplet repeat'neurodegenerative diseases are linked by a common mode of
pathogenicity, wherein a polyglutamine expansion within the relevant disease-causing
protein induces toxicity. Although details underlying pathogenesis are largely unknown,
protein chaperones appear to be effective suppressors of toxicity in various experimental
models. Understanding the protective role of chaperones might not only help us to
understand the biology of polyglutamine toxicity, but also, and perhaps more importantly …
Abstract
The ‘triplet repeat' neurodegenerative diseases are linked by a common mode of pathogenicity, wherein a polyglutamine expansion within the relevant disease-causing protein induces toxicity. Although details underlying pathogenesis are largely unknown, protein chaperones appear to be effective suppressors of toxicity in various experimental models. Understanding the protective role of chaperones might not only help us to understand the biology of polyglutamine toxicity, but also, and perhaps more importantly, inspire the design of novel therapeutic strategies.
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