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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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Detalles Bibliográficos
Autores principales: Jeng, Chung-Jiuan, Fu, Ssu-Ju, You, Chia-Ying, Peng, Yi-Jheng, Hsiao, Cheng-Tsung, Chen, Tsung-Yu, Tang, Chih-Yung
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7026490/
https://www.ncbi.nlm.nih.gov/pubmed/32117034
http://dx.doi.org/10.3389/fneur.2020.00076