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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...
Autores principales: | Fu, Ssu-Ju, Hu, Meng-Chun, Hsiao, Cheng-Tsung, Cheng, An-Ting, Chen, Tsung-Yu, Jeng, Chung-Jiuan, Tang, Chih-Yung |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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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 |
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