KCNQ1-deficient and KCNQ1-mutant human embryonic stem cell-derived cardiomyocytes for modeling QT prolongation.

ABSTRACT:

Background

The slowly activated delayed rectifier potassium current (I_Ks) mediated by the KCNQ1 gene is one of the main currents involved in repolarization. KCNQ1 mutation can result in long-QT syndrome type 1 (LQT1). I_Ks does not participate in repolarization in mice; thus, no good model is currently available for research on the mechanism of and drug screening for LQT1. In this study, we established a KCNQ1-deficient human cardiomyocyte (CM) model and performed a series of microelectrode array (MEA) detection experiments on KCNQ1-mutant CMs constructed in other studies to explore the pathogenic mechanism of KCNQ1 deletion and mutation and perform drug screening.

Method

KCNQ1 was knocked out in human embryonic stem cell (hESC) H9 line using the CRISPR/

SUBMITTER: Song Y 

PROVIDER: S-EPMC9241307 | biostudies-literature | 2022 Jun

REPOSITORIES: biostudies-literature