Article
Isogenic Sets of hiPSC-CMs Harboring Distinct KCNH2 Mutations Differ Functionally and in Susceptibility to Drug-Induced Arrhythmias.
Stem cell reports - 10 Nov 2020
Brandão Karina O, van den Brink Lettine, Miller Duncan C, Grandela Catarina, van Meer Berend J, Mol Mervyn P H, de Korte Tessa, Tertoolen Leon G J, Mummery Christine L, Sala Luca, Verkerk Arie O, Davis Richard P
Abstract excerpt
Mutations in KCNH2 can lead to long QT syndrome type 2. Variable disease manifestation observed with this channelopathy is associated with the location and type of mutation within the protein, complicating efforts to predict patient risk. Here, we demonstrated phenotypic differences in cardiomyocytes derived from isogenic human induced pluripotent stem cells (hiPSC-CMs) genetically edited to harbor mutations...
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