Dysfunction of Delayed Rectifier Potassium Channels in an Inherited Cardiac Arrhythmia

MC Sanguinetti - Annals of the New York Academy of Sciences, 1999 - Wiley Online Library
The rapid (lKr) and slow (IKs) delayed rectifier K+ currents are key regulators of cardiac
repolarization. HERG encodes the Kr channel, and KVLQT1 and hminK encode subunits …

Biophysical properties and molecular basis of cardiac rapid and slow delayed rectifier potassium channels

JS Mitcheson, MC Sanguinetti - Cellular Physiology and Biochemistry, 1999 - karger.com
Normal cardiac action potential repolarization is dependent on activation of several K+
currents, including IKr and IKs. IKr activates rapidly at positive potentials, exhibits inward …

[HTML][HTML] Properties of KvLQT1 K+ channel mutations in Romano–Ward and Jervell and Lange-Nielsen inherited cardiac arrhythmias

C Chouabe, N Neyroud, P Guicheney… - The EMBO …, 1997 - embopress.org
Mutations in the delayed rectifier K+ channel subunit KvLQT1 have been identified as
responsible for both Romano–Ward (RW) and Jervell and Lange-Nielsen (JLN) inherited …

Functional effects of mutations in KvLQT1 that cause long QT syndrome

Z Wang, M Tristani‐Firouzi, Q Xu, M Lin… - Journal of …, 1999 - Wiley Online Library
Mutations in KvLQT1. Introduction: The long QT syndrome (LQT) is caused by mutations in
genes encoding ion channels that modulate the duration of ventricular action potentials. One …

Spectrum of HERG K+-channel dysfunction in an inherited cardiac arrhythmia.

MC Sanguinetti, ME Curran… - Proceedings of the …, 1996 - National Acad Sciences
Long QT syndrome (LQT) is an autosomal dominant disorder that can cause sudden death
from cardiac arrhythmias. We recently discovered that mutations in HERG, a K+-channel …

Molecular physiology of cardiac delayed rectifier K+ channels.

MC Sanguinetti, A Zou - Heart and Vessels, 1997 - europepmc.org
Delayed rectifier K+ current in cardiac myocytes is the sum of two distinct currents, IKr and
IKs. The molecular basis of these channels has recently been defined. HERG subunits …

Short QT syndrome

R Schimpf, C Wolpert, F Gaita, C Giustetto… - Cardiovascular …, 2005 - academic.oup.com
The short QT syndrome constitutes a new clinical entity that is associated with a high
incidence of sudden cardiac death, syncope, and/or atrial fibrillation even in young patients …

Mutations in a dominant-negative isoform correlate with phenotype in inherited cardiac arrhythmias

R Mohammad-Panah, S Demolombe… - The American Journal of …, 1999 - cell.com
The long QT syndrome is characterized by prolonged cardiac repolarization and a high risk
of sudden death. Mutations in the KCNQ1 gene, which encodes the cardiac KvLQT1 …

[PDF][PDF] A mutation in HERG associated with notched T waves in long QT syndrome

E Dausse, M Berthet, I Denjoy… - Journal of molecular …, 1996 - academia.edu
Introduction gene, HERG, whose protein product likely underlies IKr, the rapidly activating
delayed rectifier current Congenital long QT syndrome (LQT) is a potentially treatable …

Pathophysiological Mechanisms of Dominant and Recessive KvLQT1 K+ Channel Mutations Found in Inherited Cardiac Arrhythmias

B Wollnik, BC Schroeder, C Kubisch… - Human molecular …, 1997 - academic.oup.com
The inherited long QT syndrome (LQTS), characterized by a prolonged QT interval in the
electrocardiogram and cardiac arrhythmia, is caused by mutations in at least four different …