Summary: Vemurafenib partially improves muscle histopathology by restoring the TGF-β/SMAD3 and mTORC1/p70S6K signaling pathways but does not improve muscle function in a mouse model of LAMA2-related-congenital muscular dystrophy. The gene discussed is LAMA2; the disease is congenital muscular dystrophy due to LMNA mutation.