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