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Loss-of-function mutations in co-chaperone BAG3 destabilize small HSPs and cause cardiomyopathy

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-...

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Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Cyhoeddwyd yn:J Clin Invest
Prif Awduron: 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
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: American Society for Clinical Investigation 2017
Pynciau:
Mynediad Ar-lein:https://ncbi.nlm.nih.gov/pmc/articles/PMC5531406/
https://ncbi.nlm.nih.gov/pubmed/28737513
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/JCI94310
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