Polycystin-1 Assembles With Kv Channels to Govern Cardiomyocyte Repolarization and Contractility.

ABSTRACT:

Background

Polycystin-1 (PC1) is a transmembrane protein originally identified in autosomal dominant polycystic kidney disease where it regulates the calcium-permeant cation channel polycystin-2. Autosomal dominant polycystic kidney disease patients develop renal failure, hypertension, left ventricular hypertrophy, and diastolic dysfunction, among other cardiovascular disorders. These individuals harbor PC1 loss-of-function mutations in their cardiomyocytes, but the functional consequences are unknown. PC1 is ubiquitously expressed, and its experimental ablation in cardiomyocyte-specific knockout mice reduces contractile function. Here, we set out to determine the pathophysiological role of PC1 in cardiomyocytes.

Methods

Wild-type and cardiomyocyte-specific PC1 knockout mice

SUBMITTER: Altamirano F 

PROVIDER: S-EPMC6733647 | biostudies-literature | 2019 Sep

REPOSITORIES: biostudies-literature