The long QT syndromes: genetic basis and clinical implications (Q33970623)

30 July 2017

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title

The long QT syndromes: genetic basis and clinical implications (English)

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30 July 2017

C E Chiang

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30 July 2017

D M Roden

series ordinal

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30 July 2017

language of work or name

English

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publication date

1 July 2000

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Journal of the American College of Cardiology

30 July 2017

published in

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30 July 2017

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30 July 2017

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30 July 2017

page(s)

1-12

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

30 July 2017

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MiRP1 forms IKr potassium channels with HERG and is associated with cardiac arrhythmia

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Heterozygous mutation in the pore of potassium channel gene KvLQT1 causes an apparently normal phenotype in long QT syndrome

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7 January 2021

The long QT syndrome: ion channel diseases of the heart

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7 January 2021

Evidence of genetic heterogeneity in the long QT syndrome

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No evidence for linkage of long QT syndrome and chromosome 11p15.5 markers in a Chinese family: evidence for genetic heterogeneity

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

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7 January 2021

KVLQT1 C-Terminal Missense Mutation Causes a Forme Fruste Long-QT Syndrome

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7 January 2021

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

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7 January 2021

Four novel KVLQT1 and four novel HERG mutations in familial long-QT syndrome

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7 January 2021

The long QT syndrome: a novel missense mutation in the S6 region of the KVLQT1 gene

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7 January 2021

Novel donor splice site mutation in the KVLQT1 gene is associated with long QT syndrome

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7 January 2021

New mutations in the KVLQT1 potassium channel that cause long-QT syndrome

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7 January 2021

Molecular genetics of the long QT syndrome: two novel mutations of the KVLQT1 gene and phenotypic expression of the mutant gene in a large kindred

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7 January 2021

A novel mutation in KVLQT1 is the molecular basis of inherited long QT syndrome in a near-drowning patient's family

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7 January 2021

Long QT syndrome-associated mutations in the S4-S5 linker of KvLQT1 potassium channels modify gating and interaction with minK subunits

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7 January 2021

Genomic organization of the KCNQ1 K+ channel gene and identification of C-terminal mutations in the long-QT syndrome.

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Molecular diagnosis of the inherited long-QT syndrome in a woman who died after near-drowning

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Inherited long QT syndromes: a paradigm for understanding arrhythmogenesis

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7 January 2021

A recessive variant of the Romano-Ward long-QT syndrome?

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7 January 2021

Recessive Romano-Ward syndrome associated with compound heterozygosity for two mutations in the KVLQT1 gene

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7 January 2021

IsK and KvLQT1: Mutation in Either of the Two Subunits of the Slow Component of the Delayed Rectifier Potassium Channel Can Cause Jervell and Lange-Nielsen Syndrome

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7 January 2021

Homozygous deletion in KVLQT1 associated with Jervell and Lange-Nielsen syndrome.

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7 January 2021

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

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7 January 2021

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7 January 2021

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7 January 2021

Missense mutation in the pore region of HERG causes familial long QT syndrome

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7 January 2021

The human delta1261 mutation of the HERG potassium channel results in a truncated protein that contains a subunit interaction domain and decreases the channel expression

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7 January 2021

Novel mechanism of HERG current suppression in LQT2: shift in voltage dependence of HERG inactivation

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7 January 2021

HERG channel dysfunction in human long QT syndrome. Intracellular transport and functional defects

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7 January 2021

Long QT syndrome-associated mutations in the Per-Arnt-Sim (PAS) domain of HERG potassium channels accelerate channel deactivation

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7 January 2021

C-terminal HERG mutations: the role of hypokalemia and a KCNQ1-associated mutation in cardiac event occurrence

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7 January 2021

Homozygous premature truncation of the HERG protein : the human HERG knockout

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7 January 2021

A mechanistic link between an inherited and an acquired cardiac arrhythmia: HERG encodes the IKr potassium channel

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7 January 2021

Novel mechanism associated with an inherited cardiac arrhythmia: defective protein trafficking by the mutant HERG (G601S) potassium channel

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7 January 2021

A minK-HERG complex regulates the cardiac potassium current I(Kr)

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7 January 2021

Class III antiarrhythmic drugs block HERG, a human cardiac delayed rectifier K+ channel. Open-channel block by methanesulfonanilides

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7 January 2021

Drug-induced torsades de pointes in one patient with congenital long QT syndrome

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7 January 2021

Phenotypic characterization of a novel long-QT syndrome mutation (R1623Q) in the cardiac sodium channel

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7 January 2021

Novel LQT-3 mutation affects Na+ channel activity through interactions between alpha- and beta1-subunits

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7 January 2021

A de novo missense mutation of human cardiac Na+ channel exhibiting novel molecular mechanisms of long QT syndrome

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7 January 2021

A de novo missense mutation (R1623Q) of the SCN5A gene in a Japanese girl with sporadic long QT sydrome. Mutations in brief no. 140. Online

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7 January 2021

Congenital long-QT syndrome caused by a novel mutation in a conserved acidic domain of the cardiac Na+ channel

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7 January 2021

Sodium channel abnormalities are infrequent in patients with long QT syndrome: identification of two novel SCN5A mutations

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7 January 2021

Genomic organization of the human SCN5A gene encoding the cardiac sodium channel

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7 January 2021

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

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7 January 2021

Molecular mechanism for an inherited cardiac arrhythmia

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7 January 2021

A single Na(+) channel mutation causing both long-QT and Brugada syndromes

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7 January 2021

Cloning of a membrane protein that induces a slow voltage-gated potassium current

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7 January 2021

K(V)LQT1 and lsK (minK) proteins associate to form the I(Ks) cardiac potassium current

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7 January 2021

Coassembly of K(V)LQT1 and minK (IsK) proteins to form cardiac I(Ks) potassium channel

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7 January 2021

Single-channel characteristics of wild-type IKs channels and channels formed with two minK mutants that cause long QT syndrome

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7 January 2021

Mutation of the gene for IsK associated with both Jervell and Lange-Nielsen and Romano-Ward forms of Long-QT syndrome

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7 January 2021

Cellular dysfunction of LQT5-minK mutants: abnormalities of IKs, IKr and trafficking in long QT syndrome

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7 January 2021

Subunit composition of minK potassium channels.

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7 January 2021

Involvement of IsK-associated K+ channel in heart rate control of repolarization in a murine engineered model of Jervell and Lange-Nielsen syndrome

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7 January 2021

Replacement by homologous recombination of the minK gene with lacZ reveals restriction of minK expression to the mouse cardiac conduction system.

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7 January 2021

A new form of long QT syndrome associated with syndactyly

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7 January 2021

Long QT syndrome associated with syndactyly identified in females

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7 January 2021

Exclusion of KCNE1 (IsK) as a Candidate Gene for Jervell and Lange-Nielsen Syndrome

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7 January 2021

Influence of pregnancy on the risk for cardiac events in patients with hereditary long QT syndrome. LQTS Investigators

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7 January 2021

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7 January 2021

The long QT syndrome in children. An international study of 287 patients

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7 January 2021

Sinus node function and ventricular repolarization during exercise stress test in long QT syndrome patients with KvLQT1 and HERG potassium channel defects.

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7 January 2021

Age-gender influence on the rate-corrected QT interval and the QT-heart rate relation in families with genotypically characterized long QT syndrome

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7 January 2021

QT dispersion: an indication of arrhythmia risk in patients with long QT intervals

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7 January 2021

Cellular basis for the normal T wave and the electrocardiographic manifestations of the long-QT syndrome

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7 January 2021

Quantitative analysis of T wave abnormalities and their prognostic implications in the idiopathic long QT syndrome.

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7 January 2021

ECG T-Wave Patterns in Genetically Distinct Forms of the Hereditary Long QT Syndrome

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7 January 2021

The long QT syndrome with impaired atrioventricular conduction: a malignant variant in infants.

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7 January 2021

Effects of verapamil and propranolol on early afterdepolarizations and ventricular arrhythmias induced by epinephrine in congenital long QT syndrome

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7 January 2021

The electrophysiological mechanism of ventricular arrhythmias in the long QT syndrome. Tridimensional mapping of activation and recovery patterns

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7 January 2021

Electrophysiological mechanism of the characteristic electrocardiographic morphology of torsade de pointes tachyarrhythmias in the long-QT syndrome: detailed analysis of ventricular tridimensional activation patterns

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7 January 2021

Diagnostic criteria for the long QT syndrome. An update

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7 January 2021

Genomic structure of three long QT syndrome genes: KVLQT1, HERG, and KCNE1.

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7 January 2021

https://api.crossref.org/works/10.1016%2FS0735-1097%2800%2900716-6

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7 January 2021

Combined use of beta-adrenergic blocking agents and long-term cardiac pacing for patients with the long QT syndrome

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7 January 2021

Dispersion of the QT interval. A marker of therapeutic efficacy in the idiopathic long QT syndrome

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7 January 2021

Cellular basis for the ECG features of the LQT1 form of the long-QT syndrome: effects of beta-adrenergic agonists and antagonists and sodium channel blockers on transmural dispersion of repolarization and torsade de pointes

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7 January 2021

Improvement of repolarization abnormalities by a K+ channel opener in the LQT1 form of congenital long-QT syndrome

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7 January 2021

The automatic implantable cardioverter-defibrillator in young patients.

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7 January 2021

Differential response to Na+ channel blockade, beta-adrenergic stimulation, and rapid pacing in a cellular model mimicking the SCN5A and HERG defects present in the long-QT syndrome

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7 January 2021

Sodium Channel Block With Mexiletine Is Effective in Reducing Dispersion of Repolarization and Preventing Torsade de Pointes in LQT2 and LQT3 Models of the Long-QT Syndrome

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7 January 2021

Long-term (subacute) potassium treatment in congenital HERG-related long QT syndrome (LQTS2)

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7 January 2021

Early Afterdepolarization Abolished by Potassium Channel Opener in a Patient with Idiopathic Long QT Syndrome

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7 January 2021

10.1016/S0735-1097(00)00716-6

Identifiers

DOI

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30 July 2017

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