This work also clarifies why deficiencies in this enzyme would be associated with loss of functional glycosylation and laminin binding of α-dystroglycan and be causal for congenital muscular dystrophy (Bao et al., 2009; Buysse et al., 2013; Czeschik et al., 2013; Shaheen et al., 2013). This evidence concerns the gene LAMB2 and congenital muscular dystrophy due to LMNA mutation.