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Publication : Assembly of multiple dystrobrevin-containing complexes in the kidney.

First Author  Loh NY Year  2000
Journal  J Cell Sci Volume  113 ( Pt 15)
Pages  2715-24 PubMed ID  10893187
Mgi Jnum  J:119852 Mgi Id  MGI:3703381
Doi  10.1242/jcs.113.15.2715 Citation  Loh NY, et al. (2000) Assembly of multiple dystrobrevin-containing complexes in the kidney. J Cell Sci 113(Pt 15):2715-24
abstractText  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 dystrophin-deficient 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: beta-dystrobrevin containing complexes are present at the basal region of renal epithelial cells, whilst alpha-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.
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