Abstract
Yazawa et al (Nature 2011;471:230–234, PMID 21307850) reprogrammed human skin cells from patients with Timothy syndrome to generate induced pluripotent stem cells and differentiated these cells into cardiomyocytes. Electrophysiologic recording and calcium (Ca2+) imaging studies of these cells revealed irregular contraction, excess Ca2+ influx, prolonged action potentials, irregular electrical activity, and abnormal calcium transients in ventricular-like cells. Roscovitine, a compound that increases the voltage-dependent inactivation of Ca(V)1.2, restored the electrical and Ca2+ signaling properties of cardiomyocytes from patients with Timothy syndrome. The authors conclude that their approach provides new opportunities for studying the molecular and cellular mechanisms of cardiac arrhythmias in humans and provides a robust assay for developing new drugs to treat these diseases.
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