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Article

Computational analysis of arrhythmogenesis in KCNH2 T618I mutation-associated short QT syndrome and the pharmacological effects of quinidine and sotalol.

NPJ systems biology and applications - 4 Nov 2022

Zhang Shugang, Lu Weigang, Yang Fei, Li Zhen, Wang Shuang, Jiang Mingjian, Wang Xiaofeng, Wei Zhiqiang

Abstract excerpt

Short QT syndrome (SQTS) is a rare but dangerous genetic disease. In this research, we conducted a comprehensive in silico investigation into the arrhythmogenesis in KCNH2 T618I-associated SQTS using a multi-scale human ventricle model. A Markov chain model of IKr was developed firstly to reproduce the experimental observations. It was then incorporated into cell, tissue, and organ models to explore how the...

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Action Potentials
Anti-Arrhythmia Agents
Arrhythmias, Cardiac
ERG1 Potassium Channel
Mutation

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PMID: 36333337
DOI: 10.1038/s41540-022-00254-5
PMCID: PMC9636227

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