Defective expression of dysferlin, a ubiquitously expressed 230-kDa transmembrane protein that has been shown to be involved in resealing muscle fiber membranes, causes limb-girdle muscular dystrophy type 2B (LGMD2B) or Miyoshi-myopathy in humans [6], [7]. The gene discussed is DYSF; the disease is autosomal recessive limb-girdle muscular dystrophy type 2B.