Vasopressin-2 receptor signaling and autosomal dominant polycystic kidney disease: from bench to bedside and back again

J Am Soc Nephrol. 2014 Jun;25(6):1140-7. doi: 10.1681/ASN.2013101037. Epub 2014 Feb 20.


Blockade of the vasopressin-2 receptor (V2R) in the kidney has recently emerged as a promising therapeutic strategy in autosomal dominant polycystic kidney disease. The pathophysiologic basis of V2R-dependent cyst proliferation and disease progression, however, is not fully understood. Recent evidence suggests that polycystic kidney disease is characterized by defects in urinary concentrating mechanisms and subsequent deregulation of vasopressin excretion by the neurohypophysis. On the cellular level, several recent studies revealed unexpected crosstalk of signaling pathways downstream of V2R activation in the kidney epithelium. This review summarizes some of the unexpected roles of V2R signaling and suggests that vasopressin signaling itself may contribute crucially to loss of polarity and enhanced proliferation in cystic kidney epithelium.

Keywords: ADPKD; Cell & Transport Physiology; Cell Signaling; collecting ducts; vasopressin.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Antidiuretic Hormone Receptor Antagonists
  • Benzazepines / therapeutic use
  • Humans
  • Kidney Tubules, Collecting / metabolism*
  • Kidney Tubules, Collecting / pathology
  • Polycystic Kidney, Autosomal Dominant / drug therapy
  • Polycystic Kidney, Autosomal Dominant / metabolism*
  • Polycystic Kidney, Autosomal Dominant / pathology
  • Receptors, Vasopressin / metabolism*
  • Signal Transduction / physiology*
  • Tolvaptan


  • AVPR2 protein, human
  • Antidiuretic Hormone Receptor Antagonists
  • Benzazepines
  • Receptors, Vasopressin
  • Tolvaptan