Pluripotent Stem Cells Model Cardiac Disease Mechanisms

Researchers generate cells from family with long-QT syndrome, generate functional myocytes

THURSDAY, July 22 (HealthDay News) -- Researchers have successfully generated pluripotent stem cells capable of recapitulating certain aspects of long-QT syndrome type 1, according to an article published online July 21 in the New England Journal of Medicine.

Alessandra Moretti, Ph.D., of the Technical University of Munich in Germany, and colleagues obtained dermal fibroblasts from two members of a family affected by long-QT syndrome type 1 and two healthy controls, infected the fibroblasts with retroviral vectors to generate pluripotent stem cells, and then directed the stem cells to differentiate into cardiac myocytes.

The researchers found that the stem cells maintained the long-QT syndrome type 1 genotype and generated functional myocytes. Action potential duration was especially prolonged in ventricular and atrial cells from the long-QT syndrome type 1 patients compared with cells from the controls. Myocytes derived from the long-QT syndrome type 1 patients had greater susceptibility to catecholamine-induced tachyarrhythmia; beta-blockade had a protective effect on this phenotype.

"We generated patient-specific pluripotent stem cells from members of a family affected by long-QT syndrome type 1 and induced them to differentiate into functional cardiac myocytes. The patient-derived cells recapitulated the electrophysiological features of the disorder," the authors write.

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