The first clue of a therapeutic approach for COL6-related myopathies came from evidence that mitochondrial dysfunction triggers the apoptosis of myofibers and medication (Irwin et al., 2003; Bernardi and Bonaldo, 2008) and that autophagy is impaired in Col6a1-/- mice as well as in UCMD patients (Grumati et al., 2010; Bernardi and Bonaldo, 2013). This evidence concerns the gene COL6A1 and myopathy.