Article
Electrophysiologic consequences of KATP gain of function in the heart: Conduction abnormalities in Cantu syndrome.
Heart rhythm - 1 Nov 2015
Levin Mark D, Zhang Haixia, Uchida Keita, Grange Dorothy K, Singh Gautam K, Nichols Colin G
Abstract excerpt
BACKGROUND: Gain-of-function (GOF) mutations in the KATP channel subunits Kir6.1 and SUR2 cause Cantu syndrome (CS), a disease characterized by multiple cardiovascular abnormalities. OBJECTIVE: The purpose of this study was to better determine the electrophysiologic consequences of such GOF mutations in the heart. METHODS: We generated transgenic mice (Kir6.1-GOF) expressing ATP-insensitive Kir6.1[G343D] subunits...
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