Fig. 1

(A) The image shows the prominent group atrophy pattern of round and angular muscle fibers with a single focus on myophagocytosis and severe endomysial fibrosis (x400). (B) The image demonstrates the loss of sarcolemmal labeling of all muscle fibers with the dysferlin (DYSF) antibody (x400) with a positive control with the β-spectrin antibody (x400). (D) The image presents the T1 mapping image and the bullseye plot, showing T1 map segmental values (mid-septal T1 value = 1040 ms). (D) The image illustrates the late enhancement images at the mid-level short-axis view and the 4-chamber view, respectively, showing no replacement fibrosis. (E) The image shows that the Leu1959 residue is highly conserved throughout species according to the multiple sequence alignment of DYSF sequences