Abstract

Protein degradation is essential to preserve cellular homeostasis since it is an important step in a variety of cellular processes. Among the intracellular proteolytic systems, the proteasome is considered to play a major role in basal protein turnover, that is, degradation of abnormal/damaged proteins. The accumulation of protein aggregates as well as various components of the ubiquitin/proteasome system raises the possibility that an impaired proteasome function may be a causal factor in the cellular degeneration that occurs in neurodegenerative disorders caused by expanded polyglutamine repeats.

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