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Assembly of multiple dystrobrevin-containing complexes in the kidney

Loh, Nellie Y., Newey, Sarah E., Davies, Kay E. and Blake, Derek J. 2000. Assembly of multiple dystrobrevin-containing complexes in the kidney. Journal of Cell Science 113 (15) , pp. 2715-2724.

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Dystrophin is the key component in the assembly and maintenance of the dystrophin-associated protein complex (DPC) in skeletal muscle. In kidney, dystroglycan, an integral component of the DPC, is involved in kidney epithelial morphogenesis, suggesting that the DPC is important in linking the extracellular matrix to the internal cytoskeleton of kidney epithelia. Here, we have investigated the molecular architecture of dystrophin-like protein complexes in kidneys from normal and dystrophindeficient mice. Using isoform-specific antibodies, we show that the different cell types that make up the kidney maintain different dystrophin-like complexes. These complexes can be broadly grouped according to their dystrobrevin content: b-dystrobrevin containing complexes are present at the basal region of renal epithelial cells, whilst a-dystrobrevin-1 containing complexes are found in endothelial and smooth muscle cells. Furthermore, these complexes are maintained even in the absence of all dystrophin isoforms. Thus our data suggest that the functions and assembly of the dystrophin-like complexes in kidney differ from those in skeletal muscle and implicate a protein other than dystrophin as the primary molecule in the assembly and maintenance of kidney complexes. Our findings also provide a possible explanation for the lack of kidney pathology in Duchenne muscular dystrophy patients and mice lacking all dystrophin isoforms.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
MRC Centre for Neuropsychiatric Genetics and Genomics (CNGG)
Subjects: R Medicine > R Medicine (General)
Uncontrolled Keywords: dystrobrevin, dystrophin, utrophin, syntrophin, Dp71, kidney, epithelium
Publisher: The Company of Biologists
ISSN: 0021-9533
Last Modified: 04 Jun 2017 05:11

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