Complement C5b-9 has been reported to be deposited on the sarcolemma of non-necrotic and cytoplasm of necrotic fibers in dysferlin-deficient mice and in patients with dysferlinopathy, making it distinct from DMD where complement C5b-9 was exclusively identified in the cytoplasm of necrotic fibers [4, 6, 7, 12, 26]. Here, DYSF is linked to neuromuscular disease caused by qualitative or quantitative defects of dysferlin.