Utilizing human induced pluripotent stem cells to study atrial arrhythmias in the short QT syndrome.

ABSTRACT:

Background

Among the monogenic inherited causes of atrial fibrillation is the short QT syndrome (SQTS), a rare channelopathy causing atrial and ventricular arrhythmias. One of the limitations in studying the mechanisms and optimizing treatment of SQTS-related atrial arrhythmias has been the lack of relevant human atrial tissues models.

Objective

To generate a unique model to study SQTS-related atrial arrhythmias by combining the use of patient-specific human induced pluripotent stem cells (hiPSCs), atrial-specific differentiation schemes, two-dimensional tissue modeling, optical mapping, and drug testing.

Methods and results

SQTS (N588K KCNH2 mutation), isogenic-control, and healthy-control hiPSCs were coaxed to differentiate into atrial cardiomyocytes using a retinoi

SUBMITTER: Shiti A 

PROVIDER: S-EPMC10589759 | biostudies-literature | 2023 Oct

REPOSITORIES: biostudies-literature