Article
Intrinsic mechanism of the enhanced rate-dependent QT shortening in the R1623Q mutant of the LQT3 syndrome.
Cardiovascular research - 1 Jan 2005
Oginosawa Yasushi, Nagatomo Toshihisa, Abe Haruhiko, Makita Naomasa, Makielski Jonathan C, Nakashima Yasuhide
Abstract excerpt
OBJECTIVE: In the type 3 long QT syndrome (LQT3), arrhythmia events tend to occur at rest or during sleep. One of the mutations, R1623Q, is located in the voltage sensor of the cardiac sodium channel (hH1), and patients with R1623Q mutation have been also reported to show bradycardia-dependent cardiac events. Although the mutant channel has been characterized by inactivation gating defects, the intrinsic...
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