Article
Hsp104 is essential for the selective degradation in yeast of polyglutamine expanded ataxin-1 but not most misfolded proteins generally.
Biochemical and biophysical research communications - 1 Jan 2010
Lee Do Hee, Goldberg Alfred L
Abstract excerpt
Molecular chaperones of the Hsp70/40 family protect against the accumulation of mutated or misfolded proteins in part by facilitating their degradation. In the polyglutamine (polyQ) diseases, mutant proteins containing expanded polyQ repeats accumulate in intracellular inclusions and cause neurodegeneration. Although the ubiquitin-proteasome system and chaperones all help protect against accumulation of such...
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