Cystic disease of the kidney.
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.
Wilson
Patricia D
PD
Division of Nephrology, Department of Medicine, Mount Sinai School of Medicine, New York, NY 10029, USA. Pat.Wilson@mssm.edu
Goilav
Beatrice
B
D000818:Animals; D017209:Apoptosis; D049109:Cell Proliferation; D004195:Disease Models, Animal; D006801:Humans; D007684:Kidney Tubules / Q000473:pathology; D051379:Mice; D009154:Mutation; D007690:Polycystic Kidney Diseases / Q000235:genetics / Q000473:pathology / Q000503:physiopathology