Chaperone activity of niflumic acid on ClC-1 chloride channel mutants causing myotonia congenita

Myotonia congenita (MC) is an inherited rare disease characterized by impaired muscle relaxation after contraction, resulting in muscle stiffness. It is caused by loss-of-function mutations in the skeletal muscle chloride channel ClC-1, important for the stabilization of resting membrane potential a...

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Bibliographic Details
Main Authors: Concetta Altamura, Elena Conte, Carmen Campanale, Paola Laghetti, Ilaria Saltarella, Giulia Maria Camerino, Paola Imbrici, Jean-François Desaphy
Format: Article
Language:English
Published: Frontiers Media S.A. 2022-08-01
Series:Frontiers in Pharmacology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fphar.2022.958196/full