Whilst the concomitant reduction of RyR1 and DHPR and the resulting excitation–contraction (EC) coupling defect are likely to explain the weakness, muscle atrophy as well as histopathological features of fiber-type disproportion and centralized nuclei are not readily explained by alterations of calcium release in recessive RYR1-related myopathies. The gene discussed is RYR1; the disease is myopathy.