Article
Gating properties of SCN5A mutations and the response to mexiletine in long-QT syndrome type 3 patients.
Circulation - 4 Sept 2007
Ruan Yanfei, Liu Nian, Bloise Raffaella, Napolitano Carlo, Priori Silvia G
Abstract excerpt
BACKGROUND: Mexiletine (Mex) has been proposed as a gene-specific therapy for patients with long-QT syndrome type 3 (LQT3) caused by mutations in the cardiac sodium channel gene (SCN5A). The degree of QT shortening and the protection from arrhythmias vary among patients harboring different mutations. We tested whether the clinical response to Mex in LQT3 could be predicted by the biophysical properties of the...
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