Spatial relationship of the C-terminal domains of dystrophin and beta-dystroglycan in cardiac muscle support a direct molecular interaction at the plasma membrane interface

Circulation Research
S StevensonN J Severs

Abstract

Dystrophin and beta-dystroglycan are components of a complex of at least nine proteins (the dystrophin-glycoprotein complex) that physically link the membrane cytoskeleton in skeletal and cardiac muscle, through the plasma membrane, to the extracellular matrix. Mutations in the dystrophin gene, which result in an absence or a quantitative or qualitative alteration of dystrophin, cause a subset of familial dilated cardiomyopathies as well as Duchenne and Becker muscular dystrophy. Biochemical studies on isolated skeletal muscle molecules indicate that dystrophin is bound to the glycoprotein complex via beta-dystroglycan, with the C-terminus of beta-dystroglycan binding to the cysteine-rich domain and first half of the C-terminal domain of dystrophin. Ultrastructural labeling has demonstrated a close spatial relationship between dystrophin and beta-dystroglycan in intact skeletal muscle, but no previous ultrastructural labeling studies have examined the dystrophin/beta-dystroglycan interaction in cardiac muscle. In the present study, we have applied complementary immunoconfocal microscopy and double immunogold fracture-label, a freeze-fracture cytochemical technique that allows high-resolution visualization of labeled membrane co...Continue Reading

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Citations

Feb 2, 2000·BioEssays : News and Reviews in Molecular, Cellular and Developmental Biology·N J Severs
Apr 4, 2008·Biological Procedures Online·Horst Robenek, Nicholas J Severs
Jun 25, 2013·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·Sang-Jun NaYoung-Chul Choi
Apr 28, 1999·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·Y S KoN J Severs
Sep 19, 2012·Pathobiology : Journal of Immunopathology, Molecular and Cellular Biology·Mara R N CelesMarcos A Rossi
Feb 17, 2001·Microscopy Research and Technique·N J SeversD Halliday
Jul 26, 2021·Analytical and Bioanalytical Chemistry·Monique G MelloDavid P Bishop

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