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Autosomal dominant polycystic disease (ADPKD) is one of the most common monogenic disorders, and globally is the third most common cause of end-stage kidney disease. Until recently, the causes of this disease remained obscure. However, the past decade has seen enormous advances in the understanding of the pathophysiology and genetics of this condition, and recent studies have suggested the possibility of specific treatment for slowing cyst growth. This review attempts to address three central questions: (i) how cysts are created; (ii) how mutated gene products give rise to cysts; and (iii) the prospects for treatment.Cysts usually develop from the collecting duct,1,,2 and are highly focal in nature, only developing in a very small proportion of nephrons. They generally increase in size and number throughout life, and encroach on normal renal tissue, causing macrophage infiltration, neovascularization, progressive fibrosis and a slow deterioration in renal function.3,,4 The disease only reaches the stage of chronic renal failure or end-stage renal disease in the fourth or fifth decade in the case of autosomal dominant polycystic disease type 1 (PKD1), or a decade later in the case of autosomal dominant polycystic disease type 2 (PKD2),5 in approximately 50% of patients.Cysts are originally connected to the mother tubule, but eventually the connection closes off, and the cysts can then only enlarge by a process of increased proliferation of mural epithelial cells and tubular secretion. In ADPKD, these cells undergo a remarkable phenotypic change, from the usual non-proliferative and reabsorptive phenotype of collecting duct principal cells, to a secretory and proliferative phenotype; this process has been extensively investigated by Grantham and coworkers.6–10 They initially microdissected cysts from polycystic kidneys, and suspended them in a hydrated collagen gel. The growth of the cysts could then be measured by … Address correspondence to Dr J. Rapoport, Department of Nephrology & Hypertension, Kaplan Medical Center, POB 1, Rehovot 76100, Israel. e-mail: jayson_r{at}clalit.org.il

Autosomal dominant polycystic kidney disease: pathophysiology and treatment