Fig. 9

Immunolocalization of proteins involved in actin dynamics. Double immunofluorescence staining was performed on serial skeletal muscle cryosections from a myotilinopathy patient (ID1 in Table 1) by using antibodies directed against MURC, MYBPH, tropomodulin 1, and CSRP3 (a Z-disc protein that is also involved in actin dynamics) and either filamin C or myotilin. Nuclei were visualized by the blue-fluorescent DAPI nucleic acid stain. Data of semiquantitative assessment of immunofluorescence intensities in abnormal areas A1–A3 are listed in Table 3. Scale bar = 50 μm