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Int. J. Mol. Sci. 2017, 18(7), 1568; doi:10.3390/ijms18071568

Proteostasis of Huntingtin in Health and Disease

Cologne Excellence Cluster for Cellular Stress Responses in Aging-Associated Diseases (CECAD), University of Cologne, Joseph Stelzmann Strasse 26, 50931 Cologne, Germany
These authors contributed equally to this work.
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Received: 14 June 2017 / Revised: 15 July 2017 / Accepted: 18 July 2017 / Published: 19 July 2017
(This article belongs to the Special Issue Neuronal Protein Homeostasis in Health and Disease)
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Abstract

Huntington’s disease (HD) is a fatal neurodegenerative disorder characterized by motor dysfunction, cognitive deficits and psychosis. HD is caused by mutations in the Huntingtin (HTT) gene, resulting in the expansion of polyglutamine (polyQ) repeats in the HTT protein. Mutant HTT is prone to aggregation, and the accumulation of polyQ-expanded fibrils as well as intermediate oligomers formed during the aggregation process contribute to neurodegeneration. Distinct protein homeostasis (proteostasis) nodes such as chaperone-mediated folding and proteolytic systems regulate the aggregation and degradation of HTT. Moreover, polyQ-expanded HTT fibrils and oligomers can lead to a global collapse in neuronal proteostasis, a process that contributes to neurodegeneration. The ability to maintain proteostasis of HTT declines during the aging process. Conversely, mechanisms that preserve proteostasis delay the onset of HD. Here we will review the link between proteostasis, aging and HD-related changes. View Full-Text
Keywords: autophagy; aging; chaperones; Huntington’s disease; neurodegeneration; proteostasis; proteasome autophagy; aging; chaperones; Huntington’s disease; neurodegeneration; proteostasis; proteasome
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Koyuncu, S.; Fatima, A.; Gutierrez-Garcia, R.; Vilchez, D. Proteostasis of Huntingtin in Health and Disease. Int. J. Mol. Sci. 2017, 18, 1568.

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