Figure S1 - Contribution of Dysferlin Deficiency to Skeletal Muscle Pathology in Asymptomatic and Severe Dystroglycanopathy Models: Generation of a New Model for Fukuyama Congenital Muscular Dystrophy

Expression of dysferlin and dysferlin-interacting proteins in fukutinHp/− mice. (A) Western blot analysis of dysferlin, caveolin-3, and MG53 in skeletal muscle extracts from fukutin-deficient fukutinHp/− (Hp/−), and control fukutinHp/+ (Hp/+) mice. A representative two individual samples for each mouse line are shown in the blots. (B) Quantification of protein expression (panel A) was shown in graphs. Data shown are the average with standard deviations (n = 4 for dysferlin, n = 3 for caveolin-3 and MG53). (C) Immunofluorescence analysis of dysferlin in fukutinHp/− (Hp/−) and fukutinHp/+ (Hp/+) mice. Bar, 50 µm. (DOCX)