GJC1 and neuromuscular disease caused by qualitative or quantitative defects of dysferlin: Since several abnormalities present in dysferlinopathies are mediated by Cx HCs and bIAJ mice with skeletal myofibers deficient in Cx43/Cx45 expression do not manifest deficient motor performance [15], it was plausible that inhibition of these channels could improve functional recovery of mice with dysferlinopathy.