Arhzaouy et al. (2019) showed that selective inactivation of VCP in skeletal muscles of Myl1p-cre-vcp-/-mice, results in a necrotic myopathy with increased macroautophagic/autophagic proteins and damaged lysosomes. It was further demonstrated that the myofiber necrosis was preceded by the upregulation of LGALS3/Galectin-3, a marker of damaged lysosomes, and TFEB activation, suggesting early defects in the lysosomal system (Arhzaouy et al., 2019). The gene discussed is VCP; the disease is myopathy.