Article
Cardiomyocytes derived from pluripotent stem cells recapitulate electrophysiological characteristics of an overlap syndrome of cardiac sodium channel disease.
Circulation - 26 Jun 2012
Davis Richard P, Casini Simona, van den Berg Cathelijne W, Hoekstra Maaike, Remme Carol Ann, Dambrot Cheryl, Salvatori Daniela, Oostwaard Dorien Ward-van, Wilde Arthur A M, Bezzina Connie R, Verkerk Arie O, Freund Christian, Mummery Christine L
Abstract excerpt
BACKGROUND: Pluripotent stem cells (PSCs) offer a new paradigm for modeling genetic cardiac diseases, but it is unclear whether mouse and human PSCs can truly model both gain- and loss-of-function genetic disorders affecting the Na(+) current (I(Na)) because of the immaturity of the PSC-derived cardiomyocytes. To address this issue, we generated multiple PSC lines containing a Na(+) channel mutation causing a...
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