Limb girdle muscular dystrophy type 2B (LGMD2B), Miyoshi myopathy, and distal myopathy are progressive, late-onset dystrophies linked to mutations in, or loss of, the dysferlin protein that present clinically as weakness in the proximal or distal muscles with elevated serum creatine kinase (CK) and muscle degeneration (Nigro, 2003; Klinge et al., 2008; Amato and Brown, 2011). The gene discussed is DYSF; the disease is Miyoshi myopathy.