Laminin α2 chain-deficient congenital muscular dystrophy, or LAMA2-CMD, is a severe form ofmuscular dystrophy caused by mutations in the LAMA2 gene.Genotype-phenotype analyses have demonstrated that complete deficiency of laminin α2 chainleads to a more severe phenotype whereas partial absence leads to a milder disease course.The clinical manifestations of complete laminin α2 chain-deficiency include profoundhypotonia at birth, widespread muscle weakness, proximal joint contractures, scoliosis anddelayed motor milestones. The gene discussed is LAMA2; the disease is congenital muscular dystrophy due to LMNA mutation.