A role for dystroglycan in basement membrane assembly

Article Abstract:

The function of the cell surface laminin receptor, dystroglycan (DG), was examined using a genetic approach. DG was previously characterized in the skeletal muscle and was considered a central element of the dystrophin-glycoprotein complex. It was discovered that DG was necessary in the development of the subendodermal basement membrane in embryoid bodies. It was also indicated that DG-laminin interactions were vital in the deposition of other basement membrane molecules.

Cell membranes, Plasma membranes, Developmental cytology, Membrane, Basement, Basement membrane

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Disruption of the sarcoglycan-sarcospan complex in vascular smooth muscle: a novel mechanism for cardiomyopathy and muscular dystrophy

Article Abstract:

A study of the pathogenesis of cardiomyopathy in relation to dystrophin-glycoprotein complex mutations was conducted. Analysis of genetically engineered mice that are deficient in either delta-sarcoglycan (Sgcd) and alpha-sarcoglycan (Sgca) revealed that only Sgcd null mice develop cardiomyopathy. The data gathered indicate that the disruption of the sarcoglycan-sarcospan complex leads to cardiomyopathy and the exacerbation of muscular dystrophy.

Genetic aspects, Cardiomyopathy, Myocardial diseases, Genetically modified mice, Muscular dystrophy

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Molecular recognition by LARGE is essential for expression of functional dystroglycan

Article Abstract:

The importance of the N-terminal domain and a portion of the mucin-like domain of alpha-dystroglycan for high-affinity laminin-receptor function are examined. The molecular recognition of dystroglycan by LARGE is the key determinant in the biosynthetic pathway for the production of mature and functional dystroglycan.

Science & research, Gene expression, Mucins

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