The most common form of dystroglycanopathy, LGMD2I, is caused by mutations in FKRP; it occurs at a frequency of 1 in 250,000 in the U.S. The use of corticosteroids to reduce fibrosis and muscle loss has shown promise with LGMD2I. Here, FKRP is linked to autosomal recessive limb-girdle muscular dystrophy type 2I.