Gating properties of SCN5A mutations and the response to mexiletine in long-QT syndrome type 3 patients

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...

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Veröffentlicht in:	Circulation (New York, N.Y.) N.Y.), 2007-09, Vol.116 (10), p.1137-1144
Hauptverfasser:	YANFEI RUAN, NIAN LIU, BLOISE, Raffaella, NAPOLITANO, Carlo, PRIORI, Silvia G
Format:	Artikel
Sprache:	eng
Schlagworte:	Adult Biological and medical sciences Blood and lymphatic vessels Cardiology. Vascular system Child Child, Preschool Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous General and cellular metabolism. Vitamins Genetic Therapy - trends Humans Infant Ion Channel Gating - drug effects Ion Channel Gating - physiology Long QT Syndrome - classification Long QT Syndrome - drug therapy Long QT Syndrome - genetics Long QT Syndrome - metabolism Medical sciences Mexiletine - pharmacology Mexiletine - therapeutic use Muscle Proteins - genetics Muscle Proteins - metabolism Mutation NAV1.5 Voltage-Gated Sodium Channel Pharmacology. Drug treatments Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Sodium Channels - genetics Sodium Channels - metabolism
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