Autosomal dominant polycystic kidney disease: molecular genetics and pathophysiology.

Abstract excerpt

In autosomal dominant polycystic kidney disease (ADPKD), the precise steps leading to cyst formation and loss of renal function remain uncertain. Pathophysiologic studies have suggested that renal tubule epithelial cells form cysts as a consequence of increased proliferation, dedifferentiation, and transition to a secretory pattern of transepithelial-fluid transport. Since the cloning of two genes implicated in...

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