The molecular biology of polycystic kidney disease

In recent years there have been a number of developments in polycystic kidn ey disease (PKD) research. The genes associated with the predominant forms of autosomal dominant PKD have been cloned, and the gene associated with a mouse model for autosomal recessive PKD has been identified and characteriz ed. Other studies have yielded new information regarding the role of the ep idermal growth factor receptor gene in promoting renal cyst formation. In t his review article we summarize recent pulished data on the molecular genet ics of autosomal dominant and autosomal recessive PKD and provide a working model of how multiple genes participate in the PKD disease pathway.