Notably, DES accumulation has been associated with mitochondrial defects in Mtm1 mouse muscles (Hnia et al., 2011), and mitochondrial defects along with triad disruption are a key aspect of the CNM disease process (Lawlor et al., 2016; Muñoz et al., 2020; Zanoteli et al., 2009). This evidence concerns the gene MTM1 and centronuclear myopathy.