To investigate contributions of dysferlin deficiency to the pathology of dystroglycanopathy, we have crossed dysferlin-deficient dysferlinsjl/sjl mice to the fukutin-knock-in fukutinHp/− and Large-deficient Largemyd/myd mice, which are phenotypically distinct models of dystroglycanopathy. Here, FKTN is linked to neuromuscular disease caused by qualitative or quantitative defects of alpha-dystroglycan.