Small molecule modulator of protein disulfide isomerase attenuates mutant huntingtin toxicity and inhibits endoplasmic reticulum stress in a mouse model of Huntington’s disease

Huntington’s disease (HD) is caused by a cytosine-adenine-guanine (CAG) trinucleotide repeat expansion in the huntingtin (HTT) gene encoding an elongated polyglutamine tract within the N-terminal of the huntingtin protein (Htt) and leads to Htt misfolding, aberrant protein aggregation, and progressi...

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Udgivet i:Hum Mol Genet
Main Authors: Zhou, Xiao, Li, Gang, Kaplan, Anna, Gaschler, Michael M, Zhang, Xiaoyan, Hou, Zhipeng, Jiang, Mali, Zott, Roseann, Cremers, Serge, Stockwell, Brent R, Duan, Wenzhen
Format: Artigo
Sprog:Inglês
Udgivet: Oxford University Press 2018
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Articles
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5905666/
https://ncbi.nlm.nih.gov/pubmed/29462355
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddy061
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