Figure 1

Immunostaining of skeletal muscle. Results of immunohistochemical examination using dystrophin, α-, β-, γ-, and δ-sarcoglycans and dystrophin antibodies are shown. There was clear staining of every protein along the plasma membrane in the control. In patient 1, γ-sarcoglycan was completely absent, and there was a mild reduction of α-sarcoglycan. Both β-sarcoglycan and δ-sarcoglycan were found to be almost entirely intact. There was clear staining of dystrophin in patient 1. In patient 2, γ-sarcoglycan was completely absent, and a patchy reduction in both α- and β-sarcoglycan was observed. Dystrophin, δ-sarcoglycan, and merosin staining patterns appeared normal.