Inward Rectifier Potassium Channels (Kir2.x) and Caveolin-3 Domain-Specific Interaction: Implications for Purkinje Cell-Dependent Ventricular Arrhythmias.

ABSTRACT:

Background

In human cardiac ventricle, I_K1 is mainly comprised Kir2.1, but Kir2.2 and Kir2.3 heterotetramers occur and modulate I_K1. Long-QT syndrome-9-associated CAV3 mutations cause decreased Kir2.1 current density, but Kir2.x heterotetramers have not been studied. Here, we determine the effect of long-QT syndrome-9-CAV3 mutation F97C on Kir2.x homo- and heterotetramers and model-associated arrhythmia mechanisms.

Methods and results

Super-resolution microscopy, co-immunoprecipitation, cellular electrophysiology, on-cell Western blotting, and simulation of Purkinje and ventricular myocyte mathematical models were used. Kir2.x isoforms have unique subcellular colocalization in human cardiomyocytes and coimmunoprecipitate with

SUBMITTER: Vaidyanathan R 

PROVIDER: S-EPMC5771420 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature