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NUB1 suppression of Huntington toxicity: mechanistic insights

Authors Yao Y, Lu B

Received 26 February 2015

Accepted for publication 1 April 2015

Published 27 May 2015 Volume 2015:5 Pages 129—136


Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 2

Editor who approved publication: Professor Nikolay Dokholyan

Yao Yao, Boxun Lu

 Department of Biophysics, School of Life Sciences, Fudan University, Shanghai, People’s Republic of China

Abstract: Huntington’s disease (HD) is an autosomal dominant neurodegenerative disorder marked by chorea, dystonia, incoordination, and cognitive and motor disturbance. The major cause of HD is the cytotoxicity of the mutant huntingtin protein (mHTT), encoded by the mutant HTT gene. The mechanism by which mHTT leads to cytotoxicity and neuronal death is unclear, and thus enhancing clearance of the mHTT protein is likely to be an effective approach to treat HD. We have recently identified NUB1 (negative regulator of ubiquitin-like proteins 1) as a modifier of mHTT levels via enhancement of its proteasomal degradation. In this review, we will discuss the mechanism of NUB1-mediated mHTT clearance and potential targeting strategies.

Keywords: drug target discovery, Huntington’s disease, NEDD8, ubiquitination

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