Cardiovascular polycystins: insights from autosomal dominant polycystic kidney disease and transgenic animal models

Delphine Bichet; Dorien Peters; Amanda Jane Patel; Patrick Delmas; Eric Honoré

doi:10.1016/j.tcm.2006.07.002

Cardiovascular polycystins: insights from autosomal dominant polycystic kidney disease and transgenic animal models

Trends Cardiovasc Med. 2006 Nov;16(8):292-8. doi: 10.1016/j.tcm.2006.07.002.

Authors

Delphine Bichet¹, Dorien Peters, Amanda Jane Patel, Patrick Delmas, Eric Honoré

Affiliation

¹ Institut de Pharmacologie Moléculaire et Cellulaire, 06560 Valbonne, France.

PMID: 17055386
DOI: 10.1016/j.tcm.2006.07.002

Abstract

Mutations in the PKD1 and PKD2 polycystin genes are responsible for autosomal dominant polycystic kidney disease (ADPKD), one of the most prevalent genetic kidney disorders. ADPKD is a multisystem disease characterized by the formation of numerous fluid-filled cysts in the kidneys, the pancreas, and the liver. Moreover, major cardiovascular manifestations are common complications in ADPKD. Intracranial aneurysms and arterial hypertension are among the leading causes of mortality in this disease. In the present review, we summarize our current understanding of the role of polycystins in the development, maintenance, and function of the cardiovascular system.

Publication types

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

MeSH terms

Animals
Cardiovascular Diseases / etiology*
Disease Models, Animal
Humans
Mice
Mice, Transgenic
Polycystic Kidney, Autosomal Dominant / etiology*
TRPP Cation Channels*

Substances

TRPP Cation Channels