Regulation of ClC-2 Chloride Channel Proteostasis by Molecular Chaperones: Correction of Leukodystrophy-Associated Defect

Regulation of ClC-2 Chloride Channel Proteostasis by Molecular Chaperones: Correction of Leukodystrophy-Associated Defect

The ClC-2 channel plays a critical role in maintaining ion homeostasis in the brain and the testis. Loss-of-function mutations in the ClC-2-encoding human <i>CLCN2</i> gene are linked to the white matter disease leukodystrophy. <i>Clcn2</i>-deficient mice display neuronal mye...

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Bibliographic Details
Main Authors: Ssu-Ju Fu, Meng-Chun Hu, Cheng-Tsung Hsiao, An-Ting Cheng, Tsung-Yu Chen, Chung-Jiuan Jeng, Chih-Yung Tang
Format: Article
Language:English
Published: MDPI AG 2021-05-01
Series:International Journal of Molecular Sciences
Subjects:
proteostasis
channelopathy
protein quality control
chaperone
co-chaperone
17-AAG
Online Access:https://www.mdpi.com/1422-0067/22/11/5859

Internet

https://www.mdpi.com/1422-0067/22/11/5859

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