CUL4-DDB1-CRBN E3 Ubiquitin Ligase Regulates Proteostasis of ClC-2 Chloride Channels: Implication for Aldosteronism and Leukodystrophy

CUL4-DDB1-CRBN E3 Ubiquitin Ligase Regulates Proteostasis of ClC-2 Chloride Channels: Implication for Aldosteronism and Leukodystrophy

Voltage-gated ClC-2 channels are essential for chloride homeostasis. Complete knockout of mouse ClC-2 leads to testicular degeneration and neuronal myelin vacuolation. Gain-of-function and loss-of-function mutations in the ClC-2-encoding human <i>CLCN2</i> gene are linked to the genetic...

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Bibliographic Details
Main Authors: Ssu-Ju Fu, Meng-Chun Hu, Yi-Jheng Peng, Hsin-Yu Fang, Cheng-Tsung Hsiao, Tsung-Yu Chen, Chung-Jiuan Jeng, Chih-Yung Tang
Format: Article
Language:English
Published: MDPI AG 2020-05-01
Series:Cells
Subjects:
channelopathy
cullin E3 ubiquitin ligase
polyubiquitination
proteasomal degradation
MG132
MLN4924
Online Access:https://www.mdpi.com/2073-4409/9/6/1332

Internet

https://www.mdpi.com/2073-4409/9/6/1332

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