Genetics, molecular mechanisms and management of long QT syndrome

An analysis of the time-relations of electrocardiograms

1920 • 3,889 citations

A mechanistic link between an inherited and an acquird cardiac arrthytmia: HERG encodes the IKr potassium channel

1995 • 2,391 citations

A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome

1995 • 2,263 citations

Positional cloning of a novel potassium channel gene: KVLQT1 mutations cause cardiac arrhythmias

1996 • 1,746 citations

Coassembly of KVLQT1 and minK (IsK) proteins to form cardiac IKS potassium channel

1996 • 1,741 citations

SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome

1995 • 1,686 citations

Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval, and sudden death

1957 • 1,593 citations

KvLQT1 and IsK (minK) proteins associate to form the IKS cardiac potassium current

1996 • 1,585 citations

HERG, a Human Inward Rectifier in the Voltage-Gated Potassium Channel Family

1995 • 1,216 citations

Effect of the Antiarrhythmic Agent Moricizine on Survival after Myocardial Infarction

1992 • 969 citations

Molecular mechanism for an inherited cardiac arrhythmia

1995 • 964 citations

The long QT syndrome. Prospective longitudinal study of 328 families.

1991 • 960 citations

A family of potassium channel genes related to eag in Drosophila and mammals.

1994 • 960 citations

The long Q-T syndrome

1975 • 901 citations

A novel mutation in the potassium channel gene KVLQT1 causes the Jervell and Lange-Nielsen cardioauditory syndrome

1997 • 878 citations

A NEW FAMILIAL CARDIAC SYNDROME IN CHILDREN.

1964 • 842 citations

Long QT Syndrome Patients With Mutations of the SCN5A and HERG Genes Have Differential Responses to Na ⁺ Channel Blockade and to Increases in Heart Rate

1995 • 801 citations

Mutations in the hminK gene cause long QT syndrome and suppress lKs function

1997 • 763 citations

The long QT syndrome

1997 • 709 citations

The Spectrum of Symptoms and QT Intervals in Carriers of the Gene for the Long-QT Syndrome

1992 • 633 citations

Multiple Mechanisms in the Long-QT Syndrome

1996 • 631 citations

Primary structure and functional expression of the human cardiac tetrodotoxin-insensitive voltage-dependent sodium channel.

1992 • 624 citations

Linkage of a Cardiac Arrhythmia, the Long QT Syndrome, and the Harvey ras -1 Gene

1991 • 526 citations

Clinical relevance of cardiac arrhythmias generated by afterdepolarizations

1994 • 483 citations

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

1996 • 465 citations

A minK–HERG complex regulates the cardiac potassium current IKr

1997 • 376 citations

Mapping of a gene for long QT syndrome to chromosome 4q25-27.

1995 • 356 citations

Molecular Basis of the Long-QT Syndrome Associated with Deafness

1997 • 350 citations

Multiple Mechanisms of Na ⁺ Channel– Linked Long-QT Syndrome

1996 • 340 citations

Cardiac sodium channel mutations in patients with long QT syndrome, an inherited cardiac arrhythmia

1995 • 337 citations

Genetically Defined Therapy of Inherited Long-QT Syndrome

1996 • 311 citations

HERG, a Primary Human Ventricular Target of the Nonsedating Antihistamine Terfenadine

1996 • 294 citations

Two long QT syndrome loci map to chromosomes 3 and 7 with evidence for further heterogeneity

1994 • 270 citations

Blockade of HERG channels expressed in Xenopus oocytes by the histamine receptor antagonists terfenadine and astemizole

1996 • 227 citations

CONGENITAL DEAFNESS ASSOCIATED WITH ELECTROCARDIOGRAPHIC ABNORMALITIES, FAINTING ATTACKS AND SUDDEN DEATH<subtitle>A Recessive Syndrome<xref ref-type="fn" rid="fn1"><sup>1</sup></xref></subtitle>

1964 • 186 citations

CONGENITAL CARDIAC ARRHYTHMIA

1964 • 133 citations

Dominant-Negative KvLQT1 Mutations Underlie the LQT1 Form of Long QT Syndrome

1997 • 131 citations

Genetical aspects of the cardio‐auditory syndrome of Jervell and Lange‐Nielsen (congenital deafness and electrocardiographic abnormalities)

1964 • 130 citations

Congenital Deaf-Mutism, Prolonged QT Interval, Syncopal Attacks and Sudden Death

1958 • 122 citations

Heritable Q-T Prolongation Without Deafness

1970 • 122 citations

Four Novel KVLQT1 and Four Novel HERG Mutations in Familial Long-QT Syndrome

1997 • 117 citations

Consistent linkage of the long-QT syndrome to the Harvey ras-1 locus on chromosome 11.

1991 • 111 citations

Proarrhythmic effects of antiarrhythmic drugs

1987 • 110 citations

Ventricular Tachyarrhythmias in the Long QT Syndromes

1984 • 93 citations

Evidence of genetic heterogeneity in Romano-Ward long QT syndrome. Analysis of 23 families.

1994 • 85 citations

A mutation in HERG Associated with Notched T waves in Long QT Syndrome

1996 • 83 citations

KVLQT1 mutations in three families with familial or sporadic long QT syndrome

1996 • 80 citations

Missense Mutation in the Pore Region of HERG Causes Familial Long QT Syndrome

1996 • 78 citations

Locus heterogeneity of autosomal dominant long QT syndrome.

1993 • 68 citations

Control of cardiac arrhythmias by lengthening repolarization

1988 • 63 citations

Polymorphism of the gene encoding a human minimal potassium ion channel (minK)

1994 • 56 citations

Inhibition of L-Type Ca ²⁺ Channel Current in Rat Ventricular Myocytes by Terfenadine

1997 • 50 citations

Torsade de pointes induced by terfenadine in a patient with long QT syndrome

1994 • 42 citations

Torsades de pointes with terfenadine ingestion

1997 • 21 citations

CONGENITAL CARDIAC ARRHYTHMIA

1964 • 21 citations

The long Q-T interval and syndromes.

1987 • 11 citations