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.
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Authors | Sovan Sarkar, David C Rubinsztein |
Journal | The FEBS journal
(FEBS J)
Vol. 275
Issue 17
Pg. 4263-70
(Sep 2008)
ISSN: 1742-464X [Print] England |
PMID | 18637946
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
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Chemical References |
- HTT protein, human
- Huntingtin Protein
- Lithium Compounds
- Nerve Tissue Proteins
- Nuclear Proteins
- Inositol
- Trehalose
- Protein Kinases
- MTOR protein, human
- TOR Serine-Threonine Kinases
- Sirolimus
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Topics |
- Autophagy
(drug effects)
- Humans
- Huntingtin Protein
- Huntington Disease
(genetics, immunology, metabolism)
- Inositol
(antagonists & inhibitors)
- Lithium Compounds
(pharmacology)
- Mutation
- Nerve Tissue Proteins
(genetics, metabolism)
- Nuclear Proteins
(genetics, metabolism)
- Protein Kinases
(metabolism)
- Sirolimus
(pharmacology)
- TOR Serine-Threonine Kinases
- Trehalose
(pharmacology)
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