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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 CLCN2 gene are linked to the white matter disease leukodystrophy. Clcn2-deficient mice display neuronal myelin vacuolation and testicular degener...

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Detalles Bibliográficos
Autores principales: Fu, Ssu-Ju, Hu, Meng-Chun, Hsiao, Cheng-Tsung, Cheng, An-Ting, Chen, Tsung-Yu, Jeng, Chung-Jiuan, Tang, Chih-Yung
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8197790/
https://www.ncbi.nlm.nih.gov/pubmed/34070744
http://dx.doi.org/10.3390/ijms22115859