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- Title
Skeletal muscle ClC-1 chloride channels in health and diseases.
- Authors
Altamura, Concetta; Desaphy, Jean-Francois; Conte, Diana; De Luca, Annamaria; Imbrici, Paola
- Abstract
In 1970, the study of the pathomechanisms underlying myotonia in muscle fibers isolated from myotonic goats highlighted the importance of chloride conductance for skeletal muscle function; 20 years later, the human ClC-1 chloride channel has been cloned; last year, the crystal structure of human protein has been solved. Over the years, the efforts of many researchers led to significant advances in acknowledging the role of ClC-1 in skeletal muscle physiology and the mechanisms through which ClC-1 dysfunctions lead to impaired muscle function. The wide spectrum of pathophysiological conditions associated with modification of ClC-1 activity, either as the primary cause, such as in myotonia congenita, or as a secondary adaptive mechanism in other neuromuscular diseases, supports the idea that ClC-1 is relevant to preserve not only for skeletal muscle excitability, but also for skeletal muscle adaptation to physiological or harmful events. Improving this understanding could open promising avenues toward the development of selective and safe drugs targeting ClC-1, with the aim to restore normal muscle function. This review summarizes the most relevant research on ClC-1 channel physiology, associated diseases, and pharmacology.
- Subjects
CHLORIDE channels; SKELETAL muscle; SKELETAL muscle physiology; PHYSIOLOGICAL adaptation; NEUROMUSCULAR diseases; PROTEIN structure; EPICATECHIN
- Publication
Pflügers Archiv: European Journal of Physiology, 2020, Vol 472, Issue 7, p961
- ISSN
0031-6768
- Publication type
Article
- DOI
10.1007/s00424-020-02376-3