Article
The Cullin 4A/B-DDB1-Cereblon E3 Ubiquitin Ligase Complex Mediates the Degradation of CLC-1 Chloride Channels.
Scientific reports - 29 May 2015
Chen Yi-An, Peng Yi-Jheng, Hu Meng-Chun, Huang Jing-Jia, Chien Yun-Chia, Wu June-Tai, Chen Tsung-Yu, Tang Chih-Yung
Abstract excerpt
Voltage-gated CLC-1 chloride channels play a critical role in controlling the membrane excitability of skeletal muscles. Mutations in human CLC-1 channels have been linked to the hereditary muscle disorder myotonia congenita. We have previously demonstrated that disease-associated CLC-1 A531V mutant protein may fail to pass the endoplasmic reticulum quality control system and display enhanced protein degradation...
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