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Curr Opin Cell Biol. 2011 Apr;23(2):190-7. doi: 10.1016/j.ceb.2010.10.010. Epub 2010 Nov 17.

Protein misfolding disorders and macroautophagy.

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1
Department of Medical Genetics, Cambridge Institute for Medical Research, University of Cambridge, Cambridge, CB 0XY, UK.

Abstract

A large group of diseases, termed protein misfolding disorders, share the common feature of the accumulation of misfolded proteins. The possibility of a common mechanism underlying either the pathogenesis or therapy for these diseases is appealing. Thus, there is great interest in the role of protein degradation via autophagy in such conditions where the protein is found in the cytoplasm. Here we review the growing evidence supporting a role for autophagic dysregulation as a contributing factor to protein accumulation and cellular toxicity in certain protein misfolding disorders and discuss the available evidence that upregulation of autophagy may be a valuable therapeutic strategy.

PMID:
21087849
PMCID:
PMC3080604
DOI:
10.1016/j.ceb.2010.10.010
[Indexed for MEDLINE]
Free PMC Article
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