eprintid: 1463877
rev_number: 60
eprint_status: archive
userid: 608
dir: disk0/01/46/38/77
datestamp: 2015-06-18 13:25:31
lastmod: 2021-10-10 22:57:06
status_changed: 2017-10-19 12:16:55
type: article
metadata_visibility: show
item_issues_count: 0
creators_name: Smith, HL
creators_name: Li, W
creators_name: Cheetham, ME
title: Molecular chaperones and neuronal proteostasis.
ispublished: pub
divisions: UCL
divisions: B02
divisions: C07
divisions: D08
keywords: Heat shock response, Molecular chaperone, Neurodegeneration, Neuroprotection, Protein aggregation, Unfolded protein response
note: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
abstract: Protein homeostasis (proteostasis) is essential for maintaining the functionality of the proteome. The disruption of proteostasis, due to genetic mutations or an age-related decline, leads to aberrantly folded proteins that typically lose their function. The accumulation of misfolded and aggregated protein is also cytotoxic and has been implicated in the pathogenesis of neurodegenerative diseases. Neurons have developed an intrinsic protein quality control network, of which molecular chaperones are an essential component. Molecular chaperones function to promote efficient folding and target misfolded proteins for refolding or degradation. Increasing molecular chaperone expression can suppress protein aggregation and toxicity in numerous models of neurodegenerative disease; therefore, molecular chaperones are considered exciting therapeutic targets. Furthermore, mutations in several chaperones cause inherited neurodegenerative diseases. In this review, we focus on the importance of molecular chaperones in neurodegenerative diseases, and discuss the advances in understanding their protective mechanisms.
date: 2015-04
official_url: http://dx.doi.org/10.1016/j.semcdb.2015.03.003
oa_status: green
full_text_type: other
language: eng
primo: open
primo_central: open_green
verified: verified_manual
elements_source: PubMed
elements_id: 1019934
doi: 10.1016/j.semcdb.2015.03.003
pii: S1084-9521(15)00046-4
lyricists_name: Cheetham, Michael
lyricists_id: MECHE52
full_text_status: public
publication: Seminars in Cell & Developmental Biology
volume: 40
pagerange: 142-152
issn: 1084-9521
citation:        Smith, HL;    Li, W;    Cheetham, ME;      (2015)    Molecular chaperones and neuronal proteostasis.                   Seminars in Cell & Developmental Biology , 40    pp. 142-152.    10.1016/j.semcdb.2015.03.003 <https://doi.org/10.1016/j.semcdb.2015.03.003>.       Green open access   
 
document_url: https://discovery.ucl.ac.uk/id/eprint/1463877/4/Cheetham.1463877__2015_accepted.pdf