Article
Loss-of-function mutations in co-chaperone BAG3 destabilize small HSPs and cause cardiomyopathy.
The Journal of clinical investigation - 1 Aug 2017
Fang Xi, Bogomolovas Julius, Wu Tongbin, Zhang Wei, Liu Canzhao, Veevers Jennifer, Stroud Matthew J, Zhang Zhiyuan, Ma Xiaolong, Mu Yongxin, Lao Dieu-Hung, Dalton Nancy D, Gu Yusu, Wang Celine, Wang Michael, Liang Yan, Lange Stephan, Ouyang Kunfu, Peterson Kirk L, Evans Sylvia M, Chen Ju
Abstract excerpt
Defective protein quality control (PQC) systems are implicated in multiple diseases. Molecular chaperones and co-chaperones play a central role in functioning PQC. Constant mechanical and metabolic stress in cardiomyocytes places great demand on the PQC system. Mutation and downregulation of the co-chaperone protein BCL-2-associated athanogene 3 (BAG3) are associated with cardiac myopathy and heart failure, and a...
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