Increased L-type calcium current causes action potential prolongation in Jervell and Lange-Nielsen syndrome and is a drug target

2025-03-21 cardiovascular medicine Title + abstract only

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BackgroundKCNQ1 loss of function variants are thought to cause type 1 long QT syndrome by reducing IKs. However, we have recently reported that pharmacologic block of IKs in human induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs) produced minimal increases in action potential duration at 90% repolarization (APD90), while genetic loss of KCNQ1 markedly prolonged APD90. We sought here to define mechanisms underlying APD prolongation by genetic loss of KCNQ1. MethodsWe studied iPSC-C...

Increased L-type calcium current causes action potential prolongation in Jervell and Lange-Nielsen syndrome and is a drug target

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