Universität zu Köln

Koyuncu, Seda, Fatima, Azra, Gutierrez-Garcia, Ricardo and Vilchez, David ORCID: 0000-0002-0801-0743 (2017). Proteostasis of Huntingtin in Health and Disease. Int. J. Mol. Sci., 18 (7). BASEL: MDPI. ISSN 1422-0067

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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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Koyuncu, Seda UNSPECIFIED UNSPECIFIED UNSPECIFIED Fatima, Azra UNSPECIFIED UNSPECIFIED UNSPECIFIED Gutierrez-Garcia, Ricardo UNSPECIFIED UNSPECIFIED UNSPECIFIED Vilchez, David UNSPECIFIED orcid.org/0000-0002-0801-0743 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-226648
DOI:	10.3390/ijms18071568
Journal or Publication Title:	Int. J. Mol. Sci.
Volume:	18
Number:	7
Date:	2017
Publisher:	MDPI
Place of Publication:	BASEL
ISSN:	1422-0067
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language UBIQUITIN-PROTEASOME SYSTEM; HEAT-SHOCK PROTEINS; EMBRYONIC STEM-CELLS; POLYGLUTAMINE-EXPANDED HUNTINGTIN; NEURONAL INTRANUCLEAR INCLUSIONS; CHAPERONE-MEDIATED AUTOPHAGY; TERMINAL MUTANT HUNTINGTIN; MOLECULAR CHAPERONES; MOUSE MODELS; NEURODEGENERATIVE DISEASE Multiple languages Biochemistry & Molecular Biology; Chemistry, Multidisciplinary Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/22664