Sarcolemma instability during mechanical activity in Largemyd cardiac myocytes with loss of dystroglycan extracellular matrix receptor function.

ABSTRACT: The abnormal glycosylation and loss of extracellular matrix receptor function of the protein dystroglycan (DG) lead to the development of muscular dystrophy and cardiomyopathy. Dystroglycan is an important receptor for extracellular matrix proteins, such as laminin, in the basement membrane surrounding muscle. Large(myd) mice have a null mutation in a gene encoding the glycosyltransferase LARGE that results in abnormal glycosylation of α-DG and phenotypes similar to those in human α-DG glycosylation-deficient muscular dystrophy. Here, we show that Large(myd) hearts with the loss of DG extracellular matrix receptor function display a cardiomyopathy characterized by myocyte damage in patches of cells positive for membrane impermeant dyes. To examine the cellular mechanisms, we show that isol

SUBMITTER: Kabaeva Z 

PROVIDER: S-EPMC3153301 | biostudies-literature | 2011 Sep

REPOSITORIES: biostudies-literature