Thus far, studies have demonstrated altered mitochondrial function in 6 LGMD subtypes (Table 4), and in one example, variations in mitochondrial-mediated apoptosis due to altered expression of the pro-survival protein BCL2 correlated with disease severity in patients homozygous for the same SGCG mutation (LGMD R5, also known as LGMD2C) [53, 147, 163, 165–169]. The gene discussed is SGCG; the disease is limb-girdle muscular dystrophy.