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Leukoencephalopathy‐causing CLCN2 mutations are associated with impaired Cl(−) channel function and trafficking

KEY POINTS: Characterisation of most mutations found in CLCN2 in patients with CC2L leukodystrophy show that they cause a reduction in function of the chloride channel ClC‐2. GlialCAM, a regulatory subunit of ClC‐2 in glial cells and involved in the leukodystrophy megalencephalic leukoencephalopathy...

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Bibliografski detalji
Izdano u:J Physiol
Glavni autori: Gaitán‐Peñas, Héctor, Apaja, Pirjo M, Arnedo, Tanit, Castellanos, Aida, Elorza‐Vidal, Xabier, Soto, David, Gasull, Xavier, Lukacs, Gergely L, Estévez, Raúl
Format: Artigo
Jezik:Inglês
Izdano: John Wiley and Sons Inc. 2017
Teme:
Online pristup:https://ncbi.nlm.nih.gov/pmc/articles/PMC5685823/
https://ncbi.nlm.nih.gov/pubmed/28905383
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1113/JP275087
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