Previous studies have shown that autophagy and endoplasmic reticulum (ER) stress are elevated in LGMD2R9 muscle (Boito et al., 2007; Franekova et al., 2021; Lin et al., 2011), suggesting that additional mechanisms, other than the impairment of α-DG glycosylation and the disruption of its interaction with laminin, may contribute to the pathogenesis of FKRP-associated dystroglycanopathies. This evidence concerns the gene LAMB2 and neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.