Until recently, it was largely thought that this muscle-specific role was related to the membrane repair activity of Dysferlin at the muscle plasma membrane, since a major pathological hallmark of Dysferlinopathies is membrane damage with an accumulation of sub-sarcolemmal membrane vesicles (Glover and Brown, 2007; Han and Campbell, 2007). Here, DYSF is linked to neuromuscular disease caused by qualitative or quantitative defects of dysferlin.