FKRP and autosomal recessive limb-girdle muscular dystrophy type 2I: Muscle biopsies from patients with LGMD2I usually show myopathic changes with reduced glycosylation of α-dystroglycan (α-DG), which probably reflects pathogenic variations in the FKRP encoding a putative Golgi-resident glycosyltransferase [8, 9].