LQT10

Long QT Syndrome Microchapters
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[1]
American Roentgen Ray Society Images of LQT10 All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]

LQT10

Type	OMIM	Mutation
LQT10		SCN4B

This novel susceptibility gene for LQT is SCN4B encoding the protein Na_Vβ4, an auxiliary subunit to the pore-forming Na_V1.5 (gene: SCN5A) subunit of the voltage-gated sodium channel of the heart. The mutation leads to a positive shift in inactivation of the sodium current, thus increasing sodium current. Only one mutation in one patient has so far been found.