Moreover, the global protein ubiquitination was significantly increased in BVES-KO muscles both before (at 2 months of age) (Supplementary Fig. 10a, b) and after the disease onset (at 8 months of age) (Fig. 5f, g), suggesting that the UPS activation is likely a direct consequence of BVES deficiency but rather a secondary event following muscular dystrophy. This evidence concerns the gene POPDC1 and muscular dystrophy.