New iPSC for old long QT syndrome modeling: putting the evidence into perspective.

Induced pluripotent stem cells (iPS cells or iPSCs) are typically derived by transfection of certain stem cell-associated genes into non-pluripotent cells, such as adult fibroblasts (typically adult somatic cells). Various diseases can be modeled through iPSC technology. The important implication of iPSCs to offer an unprecedented opportunity to recapitulate pathologic human tissue formation in vitro has generated great excitement and interest in the whole biomedical research community. Long QT syndrome (LQTS), an inherited heart disease, is characterized by prolonged QT interval on a surface electrocardiogram. LQTS presents with life-threatening cardiac arrhythmias, which can lead to fainting, syncope, and sudden death. The iPSC-derived cardiomyocytes from LQTS patients offer a potentially unlimited source of materials for biomedical study. They can be used to recapitulate complex physiological phenotypes, probe toxicological testing and drug screening, clarify the novel mechanistic insights and may also rectify gene defects at the cellular and molecular level. Despite the emerging challenges, iPSC technology has been increasingly recognized as a valuable and growing toolkit for modeling LQTS over other various models of human diseases.