Supplementary data

Supplemental Figure 1

Restoration of the sarcoglycan complex in transgenic rescue models. Sections were taken through hearts containing coronary arteries from mice of each of the following genotypes: WT (littermate control mice); dsg^–/– (δ-sarcoglycan–null mice); MHD/dsg^–/– (α-MHC–δ-sarcoglycan transgene in dsg^–/– mice); and SMD/dsg^–/– (SM22–δ-sarcoglycan transgene in dsg^–/– mice). Each section was stained with sarcoglycan Ab’s previously shown to be specific for individual sarcoglycan components: β-sarcoglycan (β-sg), γ-sarcoglycan (γ-sg), δ-sarcoglycan (δ-sg), and ζ-sarcoglycan (ζ-sg), or smooth muscle actin (sm actin). The Ab’s are indicated across the top. The presence of the α-MHC–driven MHD transgene restores cardiomyocyte sarcoglycan expression. The presence of the SM22-driven SMD transgene restores the VSM sarcoglycan complex.

Supplemental figure 1

Supplemental Figure 2

VSM sarcoglycan expression does not ameliorate pathology in sarcoglycan deficiency. Masson’s trichrome staining of hearts of gsg^–/–/SMG and dsg^–/–/SMD mice. Focal areas of degeneration and fibrosis (arrows) persist in the ventricular walls of hearts rescued for smooth muscle sarcoglycan expression. Bar: top, 1 mm; middle and bottom, 100 &mgr;m.

Supplemental figure 2

Supplemental Figure 3

Average ambulatory blood pressures of sarcoglycan-deficient transgenic mice and normal mice do not differ. Unanesthetized, ambulatory blood pressures were measured with carotid catheterization with a radiofrequency telemetry devie. Blood pressure recordings were averaged over 24–72 hours per animal and averaged among animals measured. Pulse pressure (black), systolic (dark gray), diastolic (light gray), and mean arterial pressure (white) are shown. All animal groups were not found to be significantly different from each other for all categories measured, by one-way ANOVA and Tukey’s post-test.

Supplemental figure 3