Abstract

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.

Keywords

AutophagyHuntingtinCell biologyDownregulation and upregulationMutantHuntingtin ProteinHuntington's diseaseProtein degradationCytoplasmATG16L1BiologyDiseaseChemistryBiochemistryApoptosisMedicineGene

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Publication Info

Year
2008
Type
review
Volume
275
Issue
17
Pages
4263-4270
Citations
202
Access
Closed

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Sovan Sarkar, David C. Rubinsztein (2008). Huntington’s disease: degradation of mutant huntingtin by autophagy. FEBS Journal , 275 (17) , 4263-4270. https://doi.org/10.1111/j.1742-4658.2008.06562.x

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DOI
10.1111/j.1742-4658.2008.06562.x