Limb Girdle Muscular Dystrophy 2I (LGMD2I): Is an autosomal recessive congenital muscular dystrophy caused by mutations in the gene encoding Fukutin Related Protein (FKRP) located on chromosome 19q13.2. FKRP protein is required for the glycosylation of α-dystroglycan (a component of the dystrophin associated protein complex), which is needed for binding to laminin-211/221 in the muscle basal lamina. In the brain glycosylated α-dystroglycan is required for neuronal migration during development. MDC1C is an allelic version of the disease caused by mutations on FKRP that result in muscle weakness, brain abnormalities and intellectual disability. LGMD2I patients exhibit progressive weakness in the limbs and shoulders that affect ability to walk and some patients exhibit cardiomyopathy. There is currently no cure or effective treatment or LGMD2I or MDCIC.

Targeting an increase in the α7β1 integrin laminin receptor in skeletal muscle may act to compensate for the lost laminin adhesion and reduce muscle disease progression in LGMD2I.