Sarcoglycan, delta (dystrophin-associated glycoprotein)(Sgcd) is a multimeric, integral membrane glycoprotein complex that associates with dystrophin and has been associated with inherited forms of muscular dystrophy. Mice that are homozygous for the Sgcd knock-out allele (also known as dsg-) are viable, fertile, and normal in size at weaning age. However, at 8 weeks of age there is an onset of sudden mortality, with a 50% survival rate at 28 weeks. No gene product (protein) is immunodetected in skeletal muscle microsomal preparations.&nbsp; Elevated creatine kinase serum levels are indicative of striated muscle degeneration. Histopathology of skeletal muscle tissue reveals degeneration and regeneration of muscle fibers, inflammatory infiltrate, perivascular fibrosis and calcification. At 12 weeks of age, cardiac muscle tissue also begins to show degeneration, inflammatory infiltration and perivascular fibrosis. Myofiber membranes have permeability defects as assessed by Evans blue dye uptake into myofiber cytoplasm. Skeletal muscle of mutant mice have an enhanced sensitivity to mechanically induced sarcolemmal damage. This mutant mouse strain may be useful in studies of limb girdle muscular dystrophy and cardiomyopathy.

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These Sgcd knock-out mice exhibit early mortality with elevated creatine kinase serum levels and muscle degeneration.

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