In frozen sections of skeletal muscle of the desminopathy patients or animal model, we found that desmin and VDAC1 deposition with high signals in muscle fibers, and the related pro-apoptotic proteins (bax, ATF2) also appeared high signal aggregation, and about related anti-apoptotic proteins (bcl-2, bcl-xl, HK2), HK2 in patients and bcl-2 in desminopathy rat model are slightly lower expressed than the normal ones, the other anti-apoptotic proteins are unclear. The gene discussed is DES; the disease is Desminopathy.