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

1

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PubMed ID

10898405

30 July 2017

D M Roden

series ordinal

2

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PubMed ID

10898405

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

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30 July 2017

36

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30 July 2017

1-12

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30 July 2017

1

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

30 July 2017

1 reference

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

7 January 2021

Functional distribution of right and left stellate innervation to the ventricles. Production of neurogenic electrocardiographic changes by unilateral alteration of sympathetic tone

1 reference

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

7 January 2021

Electrical alternation of the T-wave: Clinical and experimental evidence of its relationship with the sympathetic nervous system and with the long Q-T syndrome

1 reference

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

7 January 2021

The long Q-T syndrome

1 reference

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

7 January 2021

Idiopathic long QT syndrome: progress and questions

1 reference

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

7 January 2021

The long QT syndrome: a prospective international study

1 reference

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

7 January 2021

Linkage of a cardiac arrhythmia, the long QT syndrome, and the Harvey ras-1 gene

1 reference

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

7 January 2021

Locus heterogeneity of autosomal dominant long QT syndrome

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

Molecular basis of the long-QT syndrome associated with deafness.

1 reference

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

7 January 2021

KCNE1 mutations cause jervell and Lange-Nielsen syndrome

1 reference

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

7 January 2021

The spectrum of symptoms and QT intervals in carriers of the gene for the long-QT syndrome

1 reference

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

7 January 2021

Multiple mechanisms in the long-QT syndrome. Current knowledge, gaps, and future directions. The SADS Foundation Task Force on LQTS.

1 reference

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

7 January 2021

Influence of the genotype on the clinical course of the long-QT syndrome. International Long-QT Syndrome Registry Research Group.

1 reference

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

7 January 2021

Auditory stimuli as a trigger for arrhythmic events differentiate HERG-related (LQTS2) patients from KVLQT1-related patients (LQTS1).

1 reference

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

7 January 2021

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. Implications for gene-specific therapy.

1 reference

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

7 January 2021

Genetically defined therapy of inherited long-QT syndrome. Correction of abnormal repolarization by potassium

1 reference

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

7 January 2021

Low penetrance in the long-QT syndrome: clinical impact

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

The long QT syndrome: ion channel diseases of the heart

1 reference

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

7 January 2021

GENETICAL ASPECTS OF THE CARDIO-AUDITORY SYNDROME OF JERVELL AND LANGE-NIELSEN (CONGENITAL DEAFNESS AND ELECTROCARDIOGRAPHIC ABNORMALITIES).

1 reference

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

7 January 2021

Evidence of genetic heterogeneity in the long QT syndrome

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

KVLQT1 C-terminal missense mutation causes a forme fruste long-QT syndrome

1 reference

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

7 January 2021

Dominant-negative KvLQT1 mutations underlie the LQT1 form of long QT syndrome.

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

Inherited long QT syndromes: a paradigm for understanding arrhythmogenesis

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

7 January 2021

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

1 reference

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

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

Molecular mechanism for an inherited cardiac arrhythmia

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

Subunit composition of minK potassium channels.

1 reference

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

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.

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

A new form of long QT syndrome associated with syndactyly

1 reference

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

7 January 2021

Long QT syndrome associated with syndactyly identified in females

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

1 reference

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

7 January 2021

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

1 reference

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

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.

1 reference

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

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.

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

ECG T-wave patterns in genetically distinct forms of the hereditary long QT syndrome

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

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

1 reference

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

7 January 2021

Diagnostic criteria for the long QT syndrome. An update

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

7 January 2021

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

1 reference

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

7 January 2021

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

1 reference

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

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

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

The automatic implantable cardioverter-defibrillator in young patients.

1 reference

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

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

1 reference

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

7 January 2021

Sodium channel block with mexiletine is effective in reducing dispersion of repolarization and preventing torsade des pointes in LQT2 and LQT3 models of the long-QT syndrome

1 reference

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

7 January 2021

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

1 reference

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

7 January 2021

Early afterdepolarization abolished by potassium channel opener in a patient with idiopathic long QT syndrome

1 reference

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

7 January 2021

10.1016/S0735-1097(00)00716-6

Identifiers

DOI

1 reference

30 July 2017

1 reference

PubMed ID

10898405