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

Background: Autosomal dominant polycystic kidney disease is the most frequent hereditary kidney disease and is generally due to mutations in and , encoding polycystins 1 and 2. In autosomal dominant polycystic kidney disease, hypertension and cardiovascular disorders are highly prevalent, but their mechanisms are partially understood.

Methods: Since endothelial cells express the polycystin complex, where it plays a central role in the mechanotransduction of blood flow, we generated a murine model with inducible deletion of in endothelial cells (;) to specifically determine the role of endothelial polycystin-1 in autosomal dominant polycystic kidney disease.

[Cardiovascular disorders in autosomal dominant polycystic kidney disease].

Autosomal dominant polycystic kidney disease is the most frequent genetic kidney disease. Cardiovascular disorders associated with autosomal dominant polycystic kidney disease are multiple and may occur early in life. In autosomal dominant polycystic kidney disease cardiovascular morbidity and mortality are related both to the nonspecific consequences of chronic kidney disease and to the particular phenotype of autosomal dominant polycystic kidney disease.