PMID: 9529618Apr 8, 1998Paper

New insights into polycystic kidney disease and its treatment

Current Opinion in Nephrology and Hypertension
V E Torres

Abstract

Major advances in the understanding of the genetics and pathogenesis of autosomal dominant polycystic kidney disease have occurred within the past year. The proteins encoded by the PKD1 and PKD2 genes, polycystin 1 and polycystin 2, are membrane proteins, capable of interacting physically in vitro, and are likely components of a complex signalling pathway. The majority of PKD1 and PKD2 mutations so far identified are unique inactivating mutations dispersed over the entire genes. Immunohistochemical studies have shown that polycystin 1 and polycystin 2 are developmentally regulated and are overexpressed in polycystic kidneys. The cysts probably result from clonal expansions of single cells. The demonstration of loss of heterozygosity for PKD1 and the absence of immunoreactive polycystin 1 in approximately 20% of the cysts supports a two-hit tumor suppressor gene model of cystogenesis. Regardless of the nature of the initial pathogenic mechanism, the cysts in autosomal dominant polycystic kidney disease are accompanied by partial dedifferentiation of the epithelial cells, disregulation of epithelial cell proliferation, expression of a secretory phenotype, and disarray of cell matrix interactions which leads to interstitial inflam...Continue Reading

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