It is interesting that Col6a1-/- mice and patients display reduced autophagy flux [28]; reactivation of autophagy is protective against muscle mass loss in knock-out mice [28], while in BM and UCMD patients it improves the mitochondrial performance and the rate of apoptosis, downstream consequences of the autophagy defect [31]. This evidence concerns the gene COL6A1 and Ullrich congenital muscular dystrophy.