Cell Proliferation and Apoptosis in ADPKD.

Increased tubular epithelial cell proliferation with fluid secretion is a key hallmark of autosomal dominant polycystic kidney disease (ADPKD). With disruption of either PKD1 or PKD2, the main causative genes of ADPKD, intracellular calcium homeostasis and cAMP accumulation are disrupted, which in turn leads to altered signaling in the pathways that regulate cell proliferation. These dysregulations finally stimulate the development of fluid-filled cysts originating from abnormally proliferating renal tubular cells. In addition, dysregulated apoptosis is observed in dilated cystic tubules. An imbalance between cell proliferation and apoptosis seems to contribute to cyst growth and renal tissue remodeling in ADPKD. In this section, the mechanisms through which cell proliferation and apoptosis are involved in disease progression, and further, how those signaling pathways impinge on each other in ADPKD will be discussed.