Article

Dual Dysfunction of Kir2.1 Underlies Conduction and Excitation-Contraction Coupling Defects Promoting Arrhythmias in a Mouse Model of Andersen-Tawil Syndrome Type 1

2021-06-17

Abstract excerpt

<h4>ABSTRACT</h4> Andersen-Tawil Syndrome (ATS) is associated with life threatening arrhythmias of unknown mechanism. We report on a mouse model carrying the trafficking-deficient mutant Kir2.1 Δ314-315 . The mouse recapitulates the electrophysiological phenotype of type 1 (ATS1), with slower conduction velocities in response to flecainide, QT prolongation exacerbated by isoproterenol, and increased vulnerabilit...

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Literature Corpus work: caf1ad8c-1dab-5e73-88e1-d5a64e23cd5b
DOI: 10.1101/2021.06.17.448833

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