In the current study, we first aimed to verify that hypothesis by using muscle tissue from humans with MTM1 mutations and animal models of XLMTM, including the Mtm1 knockout (KO) mouse, and the well-established canine colony with a mutation in MTM1 [1, 5–7, 9, 13, 23, 38]. This evidence concerns the gene MTM1 and X-linked myotubular myopathy.