Defective Gating and Proteostasis of Human ClC-1 Chloride Channel: Molecular Pathophysiology of Myotonia Congenita

Defective Gating and Proteostasis of Human ClC-1 Chloride Channel: Molecular Pathophysiology of Myotonia Congenita

The voltage-dependent ClC-1 chloride channel, whose open probability increases with membrane potential depolarization, belongs to the superfamily of CLC channels/transporters. ClC-1 is almost exclusively expressed in skeletal muscles and is essential for stabilizing the excitability of muscle membra...

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Bibliographic Details
Main Authors: Chung-Jiuan Jeng, Ssu-Ju Fu, Chia-Ying You, Yi-Jheng Peng, Cheng-Tsung Hsiao, Tsung-Yu Chen, Chih-Yung Tang
Format: Article
Language:English
Published: Frontiers Media S.A. 2020-02-01
Series:Frontiers in Neurology
Subjects:
skeletal muscle
genetic disease
mutation
channelopathy
protein quality control
protein degradation
Online Access:https://www.frontiersin.org/article/10.3389/fneur.2020.00076/full

Internet

https://www.frontiersin.org/article/10.3389/fneur.2020.00076/full

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