TY - JOUR
T1 - Functional and therapeutic importance of purinergic signaling in polycystic kidney disease
AU - Ilatovskaya, Daria V.
AU - Palygin, Oleg
AU - Staruschenko, Alexander
N1 - Funding Information:
Research in the laboratory was partially supported by the National Heart, Lung, and Blood Institute Grant R01 HL108880, American Heart Association Grant 16EIA26720006, Juvenile Diabetes Research Foundation 1-INO-2016-223-A-N (to A. Staruschenko), Medical College of Wisconsin RAC Pilot grants (to O. Palygin and D. V. Ilatovskaya), and the National Institute of Diabetes and Digestive and Kidney Diseases Grant K99 DK105160 (to D. V. Ilatovskaya).
Publisher Copyright:
© 2016 the American Physiological Society.
PY - 2016/12/1
Y1 - 2016/12/1
N2 - 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.
AB - 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.
KW - Collecting ducts
KW - ENaC
KW - P2 receptors
KW - Purinergic signalling
KW - TRPV4
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U2 - 10.1152/ajprenal.00406.2016
DO - 10.1152/ajprenal.00406.2016
M3 - Review article
C2 - 27654892
AN - SCOPUS:85002771589
SN - 0363-6135
VL - 311
SP - F1135-F1139
JO - American Journal of Physiology - Heart and Circulatory Physiology
JF - American Journal of Physiology - Heart and Circulatory Physiology
IS - 6
ER -