The N-terminal domain of αDG, which is normally cleaved off by furin6, interacts with LARGE 24, and at least one mutation in the N-terminal domain of αDG gives rise to Limb Girdle Muscular Dystrophy where LARGE-αDG interactions are reduced along with αDG glycosylation25. Here, LARGE1 is linked to limb-girdle muscular dystrophy.