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Matthew T. Wheeler, Michael J. Allikian, Ahlke Heydemann, Michele Hadhazy, Sara Zarnegar, Elizabeth M. McNally
Published in Volume 113, Issue 5
J Clin Invest. 2004; 113(5):668–675 doi:10.1172/JCI20410
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Figure 2

Cardiomyocyte-specific transgenes rescue sarcoglycan at the membrane and restore membrane-permeability defects. Immunofluorescence microscopy was performed on sections of hearts containing coronary vessels from normal, gsg–/–, dsg–/–, gsg–/–/MHG, and dsg–/–/MHD mice at 26 weeks of age. In the left column, smooth muscle α-actin (sm actin) staining coronary VSM is shown in green; in the middle column, EBD staining is shown in red and sarcoglycan staining is shown in blue. The third column shows a merged view. (A) δ-Sarcoglycan Ab is present at the membrane of cardiomyocytes of normal and dsg–/–/MHD hearts but not dsg–/– hearts. EBD uptake (red) is found in the cardiomyocytes of dsg–/– animals but is never found in normal or dsg–/–/MHD cardiomyocytes. δ-Sarcoglycan is normally expressed in coronary arteries. Transgene expression in dsg–/–/MHD does not induce δ-sarcoglycan expression in the coronary artery tree (bottom row). (B) γ-Sarcoglycan Ab localizes γ-sarcoglycan in the membrane of normal and gsg–/–/MHG cardiomyocytes. EBD uptake (red) is seen in gsg–/– cardiomyocytes but is never seen in gsg–/–/MHG or normal cardiomyocytes. Scale bars: dsg–/– and gsg–/– are 100 μm; others are 20 μm.