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Huntington’s disease: degradation of mutant huntingtin by autophagy
Author(s) -
Sarkar Sovan,
Rubinsztein David C.
Publication year - 2008
Publication title -
the febs journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.981
H-Index - 204
eISSN - 1742-4658
pISSN - 1742-464X
DOI - 10.1111/j.1742-4658.2008.06562.x
Subject(s) - autophagy , huntingtin , microbiology and biotechnology , downregulation and upregulation , mutant , huntingtin protein , huntington's disease , protein degradation , cytoplasm , atg16l1 , biology , disease , chemistry , biochemistry , apoptosis , medicine , gene , pathology
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.