The role of chaperones in polyglutamine disease.
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.
Opal
Puneet
P
Dept of Neurology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA.
Zoghbi
Huda Y
HY
D000818:Animals; D006801:Humans; D051379:Mice; D008954:Models, Biological; D018832:Molecular Chaperones / Q000378:metabolism* / Q000502:physiology*; D019636:Neurodegenerative Diseases / Q000235:genetics* / Q000378:metabolism*; D017510:Protein Folding; D019680:Trinucleotide Repeat Expansion
D018832:Molecular Chaperones