Article
Electrophysiological mechanisms of ventricular arrhythmias in relation to Andersen-Tawil syndrome under conditions of reduced IK1: a simulation study.
American journal of physiology. Heart and circulatory physiology - 1 Dec 2006
Sung Ruey J, Wu Sheng-Nan, Wu Jiun-Shian, Chang Han-Dong, Luo Ching-Hsing
Abstract excerpt
Patients with Andersen-Tawil syndrome (ATS) mostly have mutations on the KCNJ2 gene, producing loss of function or dominant-negative suppression of the inward rectifier K(+) channel Kir2.1. However, clinical manifestations of ATS including dysmorphic features, periodic paralysis (hypo-, hyper-, or normokalemic), long QT, and ventricular arrhythmias (VAs) are considerably variable. Using a modified dynamic...
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