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]

Overview

Only one mutation in one patient of this Long QT syndrome subtype has been found.

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.