Normal desmin displayed grid-like pulp diffuse expression under sarcolemma and package (Fig 1C and S1A Fig), while desmin aggregated in muscle fiber of patients with desminopathy, which appeared the following characteristics: (1) diffuse positive aggregation within the package pulp or under sarcolemma (Fig 1C and S1A Fig); (2) strong positive lumpy deposits in the package pulp, pellet-like deposition (Fig 1C and S1A Fig); (3) strongly positive irregularly aggregates in the pulp (Fig 1C and S1A Fig). This evidence concerns the gene DES and Desminopathy.