Article
Switch From Fetal to Adult SCN5A Isoform in Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes Unmasks the Cellular Phenotype of a Conduction Disease-Causing Mutation.
Journal of the American Heart Association - 24 Jul 2017
Veerman Christiaan C, Mengarelli Isabella, Lodder Elisabeth M, Kosmidis Georgios, Bellin Milena, Zhang Miao, Dittmann Sven, Guan Kaomei, Wilde Arthur A M, Schulze-Bahr Eric, Greber Boris, Bezzina Connie R, Verkerk Arie O
Abstract excerpt
BACKGROUND: Human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) can recapitulate features of ion channel mutations causing inherited rhythm disease. However, the lack of maturity of these cells is considered a significant limitation of the model. Prolonged culture of hiPSC-CMs promotes maturation of these cells. We studied the electrophysiological effects of the I230T mutation in the sodium...
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