My laboratory uses basic science approaches to investigate clinically relevant issues. The first deals with the role of the glomerular basement membrane in kidney function and disease. We study the laminin, type IV collagen, and proteoglycan components of the basement membrane to understand how they function in glomerular filtration. We use two mouse models that serve as models for human kidney disease. Col4a3-/- mice lack the type IV collagen alpha3 chain and model Alport syndrome, or hereditary glomerulonephritis. Lamb2-/- mice lack the laminin beta2 chain and model Pierson syndrome, a form of congenital nephrotic syndrome. We are using these mice to determine how abnormalities in the glomerular basement membrane affect its intrinsic permeability and its signaling to the adjacent epithelial and endothelial cells.

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