CASQ1 and myopathy: The common molecular mechanism in these mouse models could be excessive SR leak (see the ‘Proposed pathogenic mechanism for mitochondrial damage and myopathy in CASQ1-null mice’ section below), resulting either directly by the mutations in the SR Ca2+ release channel (in RYR1Y522S/WT knock-in mice) [36,49] or indirectly by the loss of CASQ1-mediated inhibition of RYR1 channel activity in CASQ1-null mice [29,31].