The fundamental role of the ClC-1 channel has been established in congenital diseases such as Myotonia Congenita (MC) in which loss-of-function mutations in the ClC-1 gene determines sarcolemma hyperexcitability and failure of relaxation after contraction (Koch et al., 1992; Desaphy et al., 2013). This evidence concerns the gene CLCN1 and Thomsen and Becker disease.