POGLUT1 and autosomal recessive limb-girdle muscular dystrophy: POGLUT1 O-glucosylation is mainly carried out by three POGLUTs (POGLUT1, 2, and 3) that use uridine diphosphate-Glc as a substrate for modification of the EGF repetitive sequences for their O-glucosylation activities.92,93 In a family with autosomal recessive limb-girdle muscular dystrophy, a missense mutation was identified in the POGLUT1 gene.