Figure 2

Histopathological evaluation of patients with LGMD2I. (A) The pathological severity score (1 = extensive degeneration of the muscle, 2 = moderate pathological changes in morphology, 3 = mild morphological changes), reflecting the overall morphology of the muscle in terms of degeneration, regeneration, and extent of fatty fibrous-tissue infiltration, shows that there was a significant difference between the group of patients with LGMD2I homozygous (blue bar) and the group compound heterozygous (red bar) for the L276I mutation. (B-D) There was no significant difference in number of (B) necrotic fibers, (C) ring fibers or (D) internally nucleated fibers between patients homozygous (blue bar) and those compound heterozygous (red bar) for the L276I mutation. *P < 0.05.