Application of induced pluripotent stem cell technology to model long qt syndrome

Long QT syndrome (LQTS) is an arrhythmic disease associated with an increased risk of ventricular tachycardia and sudden death. Despite numerous studies that have been performed in last years, the disease is still poorly understood and existing therapy methods are not highly effective. One of the main problems is the absence of good LQTS models. Technology based on induced pluripotent stem cells (iPSCs) opens a new era in human genetic disease modeling including inherited cardiovascular diseases. Capacity of iPSCs to be differentiated into functional cardiomyocytes allows iPSC using to study molecular mechanisms of cardiovascular diseases and to search for new drugs for their therapy. In this review, application of the technology in LQTS modeling and the progress that has been made in the area will be considered in detail.