Subsequently, we have shown the importance of DNAJA3 in muscular development and mitochondrial biogenesis by establishing HSA‐Dnaja3f/f and HSA‐Dnaja3f/+ transgenic mice (mice with Dnaja3 deficiency specifically in skeletal muscle), which show severe muscular dystrophy with reduced motor activity, accompanied with impairment of activity of ATP sensor (p‐AMPK) and mitochondrial biogenesis protein, peroxisome proliferator activated receptor gamma coactivator‐1 alpha (PGC‐1α).14 Here, DNAJA3 is linked to muscular dystrophy.