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Clearance of mutant aggregate-prone proteins by autophagy.
- Source :
-
Methods in molecular biology (Clifton, N.J.) [Methods Mol Biol] 2008; Vol. 445, pp. 195-211. - Publication Year :
- 2008
-
Abstract
- The accumulation of mutant aggregate-prone proteins is a feature of several human disorders, collectively referred to as protein conformation disorders or proteinopathies. We have shown that autophagy, a cytosolic, non-specific bulk degradation system, is an important clearance route for many cytosolic toxic, aggregate-prone proteins, like mutant huntingtin and mutant alpha-synucleins. Induction of autophagy enhances the clearance of both soluble and aggregated forms of the mutant protein, and protects against toxicity caused by these mutations in cell, fly, and mouse models. Inhibition of autophagy has opposite effects. Thus, the autophagic pathway may represent a possible therapeutic target in the treatment of certain protein conformation disorders.
- Subjects :
- Animals
Blotting, Western
COS Cells
Cell Line, Tumor
Chlorocebus aethiops
Electrophoresis, Polyacrylamide Gel
Huntingtin Protein
Huntington Disease metabolism
Immunohistochemistry
Mutant Proteins genetics
Nerve Tissue Proteins genetics
Nerve Tissue Proteins metabolism
Nuclear Proteins genetics
Nuclear Proteins metabolism
Rats
Synucleins genetics
Synucleins metabolism
Autophagy physiology
Mutant Proteins metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 1064-3745
- Volume :
- 445
- Database :
- MEDLINE
- Journal :
- Methods in molecular biology (Clifton, N.J.)
- Publication Type :
- Academic Journal
- Accession number :
- 18425452
- Full Text :
- https://doi.org/10.1007/978-1-59745-157-4_13