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The 31st American Society of Nephrology meeting in Philadelphia (25-28 October 1998) provided a unique opportunity to catch up on the fast moving field of research into the pathogenesis of autosomal dominant polycystic kidney disease (ADPKD). Firstly, from the analysis of germline mutations in PKDl and PKD2 patients, the genetic basis underlying cyst formation in ADPKD is now clear. Secondly, the consistent findings of somatic PKDJ mutations in PKDl cyst epithelia is changing our concepts as to how cysts arise in the first place. Finally, the characterization of PKDl and PKD2 knockout mice has led to unexpected insights into when and how cysts arise in different tissues.

Cyst formation in ADPKD: new insights from natural and targeted mutants