Rate-dependent QT shortening mechanism for the LQT3 ΔKPQ mutant

No abstract is available for this paper.

• Publication year

  2002

• Venue

  Cardiovascular Research

• Publication date

  2002-06-01

• Fields of study

  Medicine

• Identifiers
  DOI 10.1016/S0008-6363(02)00265-1
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar

• No claims are published for this paper.

• No concepts are published for this paper.

• Inherited Brugada and Long QT-3 Syndrome Mutations of a Single Residue of the Cardiac Sodium Channel Confer Distinct Channel and Clinical Phenotypes*

  2001cited by this paper
• Two distinct congenital arrhythmias evoked by a multidysfunctional Na(+) channel.

  2000cited by this paper
• Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome.

  2000cited by this paper
• Linking a genetic defect to its cellular phenotype in a cardiac arrhythmia

  1999cited by this paper
• A single Na(+) channel mutation causing both long-QT and Brugada syndromes.

  1999cited by this paper
• Long-term follow-up of patients with long-QT syndrome treated with beta-blockers and continuous pacing.

  1999cited by this paper
• Genetic and molecular basis of cardiac arrhythmias: impact on clinical management parts I and II.

  1999cited by this paper
• Temperature dependence of early and late currents in human cardiac wild-type and long Q-T ΔKPQ Na+ channels.

  1998cited by this paper
• 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.

  1998cited by this paper
• Multiple effects of KPQ deletion mutation on gating of human cardiac Na+ channels expressed in mammalian cells.

  1998cited by this paper
• Novel LQT-3 Mutation Affects Na+ Channel Activity Through Interactions Between α- and β1-Subunits

  1998cited by this paper
• Multiple mechanisms of Na+ channel--linked long-QT syndrome.

  1996cited by this paper
• Evidence for two components of delayed rectifier K+ current in human ventricular myocytes.

  1996cited by this paper
• 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.

  1996cited by this paper
• Characterization of human cardiac Na+ channel mutations in the congenital long QT syndrome.

  1996cited by this paper
• Lidocaine block of LQT-3 mutant human Na+ channels.

  1996cited by this paper
• Assignment of the human heart tetrodotoxin-resistant voltage-gated Na+ channel alpha-subunit gene (SCN5A) to band 3p21.

  1995cited by this paper
• Molecular mechanism for an inherited cardiac arrhythmia

  1995cited by this paper
• 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.

  1995cited by this paper
• SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome.

  1995cited by this paper
• Cardiac sodium channel mutations in patients with long QT syndrome, an inherited cardiac arrhythmia.

  1995cited by this paper
• Two long QT syndrome loci map to chromosomes 3 and 7 with evidence for further heterogeneity

  1994cited by this paper
• Effects of III-IV linker mutations on human heart Na+ channel inactivation gating.

  1994cited by this paper
• Efficacy of Permanent Pacing in the Management of High‐Risk Patients With Long QT Syndrome

  1991cited by this paper
• Early Afterdepolarizations: Mechanism of Induction and Block A Role for L‐Type Ca2+ Current

  1989cited by this paper
• Sodium current in voltage clamped internally perfused canine cardiac Purkinje cells.

  1987cited by this paper
• Effect of Tetrodotoxin on Membrane Currents in Mammalian Cardiac Fibres

  1967cited by this paper
• Life-threatening Arrhythmias Genotype-phenotype Correlation in the Long-qt Syndrome : Gene-specific Triggers for Genotype-phenotype Correlation in the Long-qt Syndrome Gene-specific Triggers for Life-threatening Arrhythmias

  year unknowncited by this paper

• Modeling Genetic Cardiac Channelopathies Using Induced Pluripotent Stem Cells - Status quo from an Electrophysiological Perspective.

  2021cites this paper
• Late sodium current and calcium homeostasis in arrhythmogenesis

  2020cites this paper
• Late Sodium Current Inhibitors as Potential Antiarrhythmic Agents

  2020cites this paper
• Hemodynamic Effects of Late Sodium Current Inhibitors in a Swine Model of Heart Failure.

  2019cites this paper
• Ion channels, long QT syndrome and arrhythmogenesis in ageing

  2017cites this paper
• Mechanisms of Mutation-Specific Inhibition of Late Na+ Current in Long QT Syndrome Type 3

  2017cites this paper
• Molecular Pathophysiology of Congenital Long QT Syndrome.

  2017cites this paper
• Late INa Inhibition as an Antiarrhythmic Strategy

  2017cites this paper
• Murine Electrophysiological Models of Cardiac Arrhythmogenesis.

  2017cites this paper
• QT Dynamics During Exercise in Asymptomatic Children with Long QT Syndrome Type 3

  2016cites this paper
• Late INa in the Heart

  2015cites this paper
• Intracellular Calcium Attenuates Late Current Conducted by Mutant Human Cardiac Sodium Channels

  2015cites this paper
• Late I(Na) in the Heart: Physiology, Pathology, and Pathways.

  2015cites this paper
• 98 – Sudden Infant Death Syndrome

  2014cites this paper
• Light phase-restricted feeding slows basal heart rate to exaggerate the type-3 long QT syndrome phenotype in mice.

  2014cites this paper
• The arrhythmogenic consequences of increasing late INa in the cardiomyocyte.

  2013cites this paper
• Arrhythmogenic left atrial cellular electrophysiology in a murine genetic long QT syndrome model.

  2011cites this paper
• The common African American polymorphism SCN5A-S1103Y interacts with mutation SCN5A-R680H to increase late Na current.

  2011cites this paper
• Late Sodium Current Contributes to the Reverse Rate-Dependent Effect of IKr Inhibition on Ventricular Repolarization

  2011cites this paper
• Discontinuous conduction through heterogeneous cardiac junctions

  2011cites this paper
• History of Basic Science in Cardiac Electrophysiology

  2011cites this paper
• Autonomic modulation and antiarrhythmic therapy in a model of long QT syndrome type 3

  2010cites this paper
• Loss-of-function mutation of the SCN3B-encoded sodium channel {beta}3 subunit associated with a case of idiopathic ventricular fibrillation.

  2010cites this paper
• Using computational modeling to predict arrhythmogenesis and antiarrhythmic therapy.

  2009cites this paper
• Differential modulation of late sodium current by protein kinase A in R1623Q mutant of LQT3.

  2009cites this paper
• Molecular and Clinical Characterization of a Novel SCN5A Mutation Associated With Atrioventricular Block and Dilated Cardiomyopathy

  2008cites this paper
• A novel C-terminal truncation SCN5A mutation from a patient with sick sinus syndrome, conduction disorder and ventricular tachycardia.

  2007cites this paper
• Inherited Arrhythmias: A National Heart, Lung, and Blood Institute and Office of Rare Diseases Workshop Consensus Report About the Diagnosis, Phenotyping, Molecular Mechanisms, and Therapeutic Approaches for Primary Cardiomyopathies of Gene Mutations Affecting Ion Channel Function

  2007cites this paper
• [Sudden infant death syndrome].

  2007cites this paper
• Characterization of the cardiac sodium channel SCN5A mutation, N1325S, in single murine ventricular myocytes.

  2007cites this paper
• Altered Na+ Channels Promote Pause-Induced Spontaneous Diastolic Activity in Long QT Syndrome Type 3 Myocytes

  2006cites this paper
• Action Potential Duration, Rate of Stimulation, and Intracellular Sodium

  2006cites this paper
• Partial expression defect for the SCN5A missense mutation G1406R depends on splice variant background Q1077 and rescue by mexiletine.

  2006cites this paper
• Long‐QT syndrome‐related sodium channel mutations probed by the dynamic action potential clamp technique

  2006cites this paper
• Repolarization Reserve in Cardiac Cells

  2006cites this paper
• Preclinical cardiac safety assessment of pharmaceutical compounds using an integrated systems-based computer model of the heart.

  2006cites this paper
• Abnormalities of inward currents underlying cardiac depolarization: role in acquired and heritable arrhythmias

  2005cites this paper
• Intrinsic mechanism of the enhanced rate-dependent QT shortening in the R1623Q mutant of the LQT3 syndrome.

  2005cites this paper
• Mechanisms by which SCN5A mutation N1325S causes cardiac arrhythmias and sudden death in vivo.

  2004cites this paper
• A trafficking defective, Brugada syndrome-causing SCN5A mutation rescued by drugs.

  2004cites this paper
• Chapter 15 – Pharmacology of the Cardiac Sodium Channel

  2004cites this paper
• Stimulation of Protein Kinase C Inhibits Bursting in Disease-Linked Mutant Human Cardiac Sodium Channels

  2003cites this paper
• A Ubiquitous Splice Variant and a Common Polymorphism Affect Heterologous Expression of Recombinant Human SCN5A Heart Sodium Channels

  2003cites this paper
• A common human SCN5A polymorphism modifies expression of an arrhythmia causing mutation.

  2003cites this paper
• Genetic control of sodium channel function.

  2003cites this paper
• Effect of pacing and mexiletine on dispersion of repolarisation and arrhythmias in DeltaKPQ SCN5A (long QT3) mice.

  2003cites this paper