Fig. 2

Muscle pathology A-C LGMDR2: (A) Increased fibers with internalized nuclei (HE staining); (B) Typical ragged red fibers (MGT staining, arrows); (C) Significantly reduced dysferlin immunostaining compared with the contemporaneous normal control. D-F LGMDR1: (D) Eosinophils scattered in the endomysium (HE staining, arrows); (E) Typical whorled fibers (NADH staining, arrows). (F) Negative MHC-I expression. G-I IIM: (G) Dysferlin immunostaining was also significantly reduced in anti-HMGCR patients. (H) MAC deposition on capillaries could be detected in an MSA-negative IIM patient (arrows). (I) Diffuse MHC-I expression in an anti-HMGCR-positive IIM patient