LQT4

Long QT Syndrome Microchapters
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[1]
American Roentgen Ray Society Images of LQT4 All Images X-rays Echo & Ultrasound CT Images MRI
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]

LQT4 Subtype

Type	OMIM	Mutation	Notes
LQT4	600919	anchor protein Ankyrin B	LQT4 is very rare. Ankyrin B anchors the ion channels in the cell.

The LQT4 genes are ANK2 and ANKB, and code for proteins called ankyrins. They are proteins which bind to several important ion channel proteins such as the chloride-bicarbonate anion exchanger, ATPase, calcium release channels, and the voltage gated sodium channel. A mutation in the LTQ4 genes that code for ankyrins can cause increased intracellular concentrations of calcium, and can therefore cause fatal arrhythmias.