Article
Long QT syndrome: ionic basis and arrhythmia mechanism in long QT syndrome type 1.
Journal of cardiovascular electrophysiology - 1 Jun 2000
Sanguinetti M C
Abstract excerpt
Long QT syndrome type 1 (LQT1) causes torsades de pointes arrhythmia, ventricular fibrillation, and sudden death. It usually is inherited as an autosomal dominant trait (Romano-Ward syndrome). The primary defect in LQT1 is a mutation in KVLQT1, a gene that encodes the pore-forming alpha-subunit of a K+ channel. KvLQT1 alpha-subunits coassemble with minK beta-subunits to form channels that conduct the slow delayed...
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