Huntington's disease: degradation of mutant huntingtin by autophagy

S Sarkar, DC Rubinsztein - The FEBS journal, 2008 - Wiley Online Library
The FEBS journal, 2008Wiley Online Library
Autophagy is a nonspecific bulk degradation pathway for long‐lived cytoplasmic proteins,
protein complexes, or damaged organelles. This process is also a major degradation
pathway for many aggregate‐prone, disease‐causing proteins associated with
neurodegenerative disorders, such as mutant huntingtin in Huntington's disease. In this
review, we discuss factors regulating the degradation of mutant huntingtin by autophagy. We
also report the growing list of new drugs/pathways that upregulate autophagy to enhance …
Autophagy is a nonspecific bulk degradation pathway for long‐lived cytoplasmic proteins, protein complexes, or damaged organelles. This process is also a major degradation pathway for many aggregate‐prone, disease‐causing proteins associated with neurodegenerative disorders, such as mutant huntingtin in Huntington’s disease. In this review, we discuss factors regulating the degradation of mutant huntingtin by autophagy. We also report the growing list of new drugs/pathways that upregulate autophagy to enhance the clearance of this mutant protein, as autophagy upregulation may be a tractable strategy for the treatment of Huntington’s disease.
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