Fig. 5

Immunolocalization of Z-disc (−associated) proteins I. Serial skeletal muscle cryosections from a myotilinopathy patient (ID1 in Table 1) were double-stained with antibodies recognizing the indicated Z-disc (−associated) proteins and either filamin C or myotilin as a positive control to localize abnormal fibers. Nuclei were visualized by DAPI staining. Results of semiquantitative evaluation of signal intensities in the different abnormal areas A1–A3 are specified in Table 3. A1: abnormal area 1; A2: abnormal area 2; A3: abnormal area 3. Scale bar = 50 μm