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Annu Rev Pathol. 2007;2:341-68.

Cystic disease of the kidney.

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1
Division of Nephrology, Department of Medicine, Mount Sinai School of Medicine, New York, NY 10029, USA. Pat.Wilson@mssm.edu

Abstract

This review focuses on the mechanisms that underlie the development of human renal cystic diseases. A pathological, clinical, and pathophysiological overview is given. Initial analysis of the cell biology of inappropriate hyperproliferation accompanied by fluid secretion of cyst-lining epithelia has been followed by the elucidation of fundamental defects in epithelial polarity, cell-matrix and cell-cell interactions, and apoptosis, all of which are discussed. Identification of the genes and proteins responsible for several renal cystic diseases has led to a more complete understanding of defects in renal developmental programming, differentiation, and morphogenesis, all of which underlie cystic diseases of the kidney.

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