Long QT Syndrome-associated Mutations in the Per-Arnt-Sim (PAS) Domain of HERG Potassium Channels Accelerate Channel Deactivation
Population
Oocyte expression system expressing eight long QT syndrome-associated mutations in the amino-terminal region…
Design
Preclinical
Authors
Discussion
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Implication
May underlie QT prolongation in LQTS2 patients; extends HERG gating insights but leaves open human translation.
Structured PICO
P
Population
Oocyte expression system expressing eight long QT syndrome-associated mutations in the amino-terminal region of HERG (F29L, N33T, G53R, R56Q, C66G, H70R, A78P, and L86R)
I
Intervention
Expression of mutant HERG subunits
O
Outcome
Channel gating properties including deactivation rate and voltage dependence of channel availabilitysurrogate
Mutations in the PAS domain of HERG channels accelerate deactivation, providing a mechanistic basis for reduced outward potassium current and QTc prolongation in long QT syndrome.
Cite This Study
Zou et al. (1999) studied this question.