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Endothelium-Specific Deficiency of Polycystin-1 Promotes Hypertension and Cardiovascular Disorders

Mouad Hamzaoui1,2, Deborah Groussard1, Dorian Nezam1,2

  • 1Normandie Univ, UNIROUEN, INSERM U1096, Rouen, France (M.H., D.G., D.N., Z.D., G.L., V.T., A.D., S.R., P.M., V.R., J.B., D.G.).

Hypertension (Dallas, Tex. : 1979)
|September 12, 2022
PubMed

Insights

Loss of polycystin-1 in endothelial cells causes hypertension and vascular dysfunction, worsening chronic kidney disease complications. This study highlights polycystin-1

Area of Science:

  • Nephrology
  • Cardiovascular Biology
  • Genetics

Background:

  • Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease, often caused by mutations in PKD1 and PKD2.
  • Hypertension and cardiovascular issues are common in ADPKD, but their underlying mechanisms are not fully understood.

Purpose of the Study:

  • To investigate the specific role of endothelial polycystin-1 in ADPKD and related cardiovascular complications.
  • To determine if endothelial polycystin-1 is essential for normal blood flow mechanotransduction and vascular development.

Main Methods:

  • Generated a mouse model with inducible deletion of Pkd1 in endothelial cells (Cdh5-CreERT2;Pkd1fl/fl).
  • Assessed endothelial function, blood pressure, arteriovenous fistula development, and cardiovascular changes in both normal and chronic kidney disease models.

Main Results:

  • Endothelial Pkd1 deletion led to endothelial dysfunction, characterized by impaired vasodilation and increased blood pressure.
  • Deletion of endothelial Pkd1 hindered arteriovenous fistula development and exacerbated vascular remodeling and heart hypertrophy in a chronic kidney disease model.

Conclusions:

  • This study provides the first in vivo evidence that endothelial polycystin-1 is crucial for maintaining vascular health.
  • Loss of endothelial polycystin-1 promotes endothelial dysfunction, hypertension, and worsens cardiovascular complications in the context of kidney disease.
Abstract

Keywords:
autosomal dominant polycystic kidney diseasechronic kidney diseaseciliopathiesendothelial dysfunctionhypertensionpolycystin

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