Functional and therapeutic importance of purinergic signaling in polycystic kidney disease.

Polycystic kidney diseases (PKD) are a group of inherited nephropathies marked with the formation of fluid-filled cysts along the nephron. This renal disorder affects millions of people worldwide, but current treatment strategies are unfortunately limited to supportive therapy, dietary restrictions, and, eventually, renal transplantation. Recent advances in PKD management are aimed at targeting exaggerated cell proliferation and dedifferentiation to interfere with cyst growth. However, not nearly enough is known about the ion transport properties of the cystic cells, or specific signaling pathways modulating channels and transporters in this condition. There is growing evidence that abnormally elevated concentrations of adenosine triphosphate (ATP) in PKD may contribute to cyst enlargement; change in the profile of purinergic receptors may also result in promotion of cystogenesis. The current mini-review is focused on the role of ATP and associated signaling affecting ion transport properties of the renal cystic epithelia.