LQT1


Also found in: Acronyms.

KCNQ1

A gene on chromosome 11p15.5 that encodes a voltage-gated potassium channel required for the repolarisation phase of the cardiac action potential. The KCNQ1 gene product can form heteromultimers with two other potassium channel proteins, KCNE1 and KCNE3.
 
Molecular pathology
KCNQ1 mutations are associated with hereditary long QT syndrome/Romano-Ward syndrome, Jervell and Lange-Nielsen syndrome and familial atrial fibrillation.

LQT1

, LQT2, LQT3 Molecular medicine Three genes, located on chromosomes 11, 7, and 3 respectively, that have been linked to the long QT syndrome. See Long QT syndrome.
References in periodicals archive ?
Delayed rectifier currents were measured in specific ventricular-like cells: the cardiomyocytes (CM) derived from patient-specific LQT1 iPS cells showed reduced [I.sub.Ks] peak and tail current densities, whereas [I.sub.Kr] conductance appeared to be regular.
[beta]-blockers can reduce the overall amount of cardiac events in LQT2 patients but they induce more cardiac events compared to the LQT1 collective [30-33].
Antzelevitch, "Differential effects of beta-adrenergic agonists and antagonist in LQT1, LQT2 and LQT3 models of the long QT syndrome," Journal of the American College of Cardiology, vol.
Kurita et al., "Gene-specific response of dynamic ventricular repolarization to sympathetic stimulation in LQT1, LQT2 and LQT3 forms of congenital long QT syndrome," European Heart Journal, vol.
In LQT1, however, these conditions result in relatively limited prolongation and no EADs.
The mainstay of therapy in patients with LQTS remains beta-blockers, particularly in LQT1 patients.
Positional cloning[31] was used to identify the LQT1 gene on chromosome 11p15.5, the gene initially localized by Keating et al.[20,22] This gene was found to be a novel potassium channel gene, initially called KVLQT1 and later renamed KCNQ1.
Since mutations in KVLQT1 were shown to cause LQTS (LQT1), mutations in minK were sought because mink plays an essential role in the development of [I.sub.Ks] (see "LQT5: minK").[36]
For instance, they showed that mutations in LQT1 and LQT2 result in early symptoms (ie, syncope), but the risk of sudden death was relatively low for any event.