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Abnormal development of glomerular endothelial and mesangial cells in mice with targeted disruption of the lama3 gene.

Kidney Int

September 2006

Department of Medicine, Division of Gerontology and Geriatric Medicine, University of Washington School of Medicine, University of Washington Medicine at South Lake Union, Seattle, Washington, USA.

Mice with targeted disruption of the lama3 gene, which encodes the alpha3 chain of laminin-5 (alpha3beta3gamma2, 332), develop a blistering skin disease similar to junctional epidermolysis bullosa in humans. These animals also develop abnormalities in glomerulogenesis. In both wild-type and mutant animals (lama3(-/-)), podocytes secrete glomerular basement membrane and develop foot processes.

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