Novel LQT-3 Mutation Affects Na+ Channel Activity Through Interactions Between α- and β1-Subunits

No abstract is available for this paper.

• Publication year

  1998

• Venue

  Circulation Research

• Publication date

  1998-07-27

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1161/01.RES.83.2.141
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar

• No claims are published for this paper.

• No concepts are published for this paper.

• A new glucose 6 phosphate dehydrogenase variant, G6PD Sinnai (34 G→T)

  1998cited by this paper
• Pharmacological targeting of long QT mutant sodium channels.

  1997cited by this paper
• Mutations in the hminK gene cause long QT syndrome and suppress lKs function

  1997cited by this paper
• The newborn rabbit sino‐atrial node expresses a neuronal type I‐like Na+ channel.

  1997cited by this paper
• Positional cloning of a novel potassium channel gene: KVLQT1 mutations cause cardiac arrhythmias

  1996cited by this paper
• Pathophysiology of ion channel mutations.

  1996cited by this paper
• Coexpression of beta 1 with cardiac sodium channel alpha subunits in oocytes decreases lidocaine block.

  1996cited by this paper
• Molecular properties of sodium and calcium channels

  1996cited by this paper
• Characterization of a TTX-sensitive Na+ current in pacemaker cells isolated from rabbit sinoatrial node.

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

  1996cited by this paper
• Molecular Determinants of β1 Subunit-Induced Gating Modulation in Voltage-Dependent Na+ Channels

  1996cited by this paper
• Genetically defined therapy of inherited long-QT syndrome. Correction of abnormal repolarization by potassium.

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

  1996cited by this paper
• The long QT syndrome: new diagnostic and therapeutic approach in the era of molecular biology.

  1996cited by this paper
• Multiple mechanisms of Na+ channel--linked long-QT syndrome.

  1996cited by this paper
• Functional association of the beta 1 subunit with human cardiac (hH1) and rat skeletal muscle (mu 1) sodium channel alpha subunits expressed in Xenopus oocytes

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

  1995cited by this paper
• Modulation of cardiac Na+ channel expression in Xenopus oocytes by beta 1 subunits.

  1995cited by this paper
• Modification of inactivation in cardiac sodium channels: ionic current studies with Anthopleurin-A toxin

  1995cited by this paper
• Modulation of Cardiac Na+ Channel Expression in Xenopus Oocytes by β1 Subunits (*)

  1995cited by this paper
• Mapping of a gene for long QT syndrome to chromosome 4q25-27.

  1995cited by this paper
• Voltage-dependent open-state inactivation of cardiac sodium channels: gating current studies with Anthopleurin-A toxin

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

  1995cited by this paper
• A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome.

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

  1995cited by this paper
• Voltage-gated Na+ channel beta 1 subunit mRNA expressed in adult human skeletal muscle, heart, and brain is encoded by a single gene.

  1994cited by this paper
• Na+ channels must deactivate to recover from inactivation.

  1994cited by this paper
• Expression of the sodium channel beta 1 subunit in rat skeletal muscle is selectively associated with the tetrodotoxin-sensitive alpha subunit isoform.

  1993cited by this paper
• Structure and function of voltage-gated ion channels.

  1993cited by this paper
• Evidence of genetic heterogeneity in the long QT syndrome.

  1993cited by this paper
• Partial characterization of the rH1 sodium channel protein from rat heart using subtype-specific antibodies.

  1993cited by this paper
• Correlation between electrical activity and the size of rabbit sino‐atrial node cells.

  1993cited by this paper
• Expression of the sodium channel β1 subunit in rat skeletal muscle is selectively associated with the tetrodotoxin-sensitive α subunit isoform

  1993cited by this paper
• Primary structure and functional expression of the human cardiac tetrodotoxin-insensitive voltage-dependent sodium channel.

  1992cited by this paper
• Primary structure and functional expression of the beta 1 subunit of the rat brain sodium channel.

  1992cited by this paper
• The Long QT Syndrome: Prospective Longitudinal Study of 328 Families

  1991cited by this paper
• Molecular kinetics of voltage-dependent Na+ channels.

  1991cited by this paper
• Sodium channel inactivation from resting states in guinea‐pig ventricular myocytes.

  1991cited by this paper
• Beta 1 subunits of sodium channels. Studies with subunit-specific antibodies.

  1990cited by this paper
• Electrocardiographic quantitation of ventricular repolarization.

  1989cited by this paper
• The voltage-sensitive sodium channel from rabbit skeletal muscle. Chemical characterization of subunits.

  1987cited by this paper
• [Long Q-T syndrome].

  1986cited by this paper
• Local anesthetics: hydrophilic and hydrophobic pathways for the drug- receptor reaction

  1977cited by this paper
• Evidence of Genetic Heterogeneity in the Long QT Syndrome

  year unknowncited by this paper

• Pediatric flecainide pharmacogenomics: a roadmap to delivering precision-based care to pediatrics arrhythmias

  2024cites this paper
• Biophysical mechanisms of myocardium sodium channelopathies

  2024cites this paper
• Beta-subunit-eliminated eHAP expression (BeHAPe) cells reveal subunit regulation of the cardiac voltage-gated sodium channel

  2023cites this paper
• Cardiac sodium channel complexes and arrhythmia: structural and functional roles of the β1 and β3 subunits

  2022cites this paper
• Physiology and Molecular Biology of Ion Channels Underlying Ventricular Repolarization of the Mammalian Heart

  2019cites this paper
• Structural basis of α-scorpion toxin action on Nav channels

  2019cites this paper
• Detection of Nav1.5 Conformational Change in Mammalian Cells Using the Noncanonical Amino Acid ANAP.

  2019cites this paper
• Mutations in NaV1.5 Reveal Calcium-Calmodulin Regulation of Sodium Channel

  2019cites this paper
• The voltage-gated sodium channel EF-hands form an interaction with the III-IV linker that is disturbed by disease-causing mutations

  2018cites this paper
• Development of a genetically encoded site-specific fluorescent sensor of human cardiac voltage-gated sodium channel inactivation

  2018cites this paper
• Mechanisms of noncovalent β subunit regulation of NaV channel gating

  2017cites this paper
• Multi-scale modeling and variability in cardiac cellular electrophysiology

  2017cites this paper
• Calmodulin limits pathogenic Na+ channel persistent current

  2017cites this paper
• Using fluorescence to understand β subunit–Na V channel interactions

  2017cites this paper
• Using fluorescence to understand β subunit–NaV channel interactions

  2017cites this paper
• Cardiac Sodium Channel Mutations: Why so Many Phenotypes?

  2016cites this paper
• Cardiac Na Channels: Structure to Function.

  2016cites this paper
• Myocardial KChIP2 Expression in Guinea Pig Resolves an Expanded Electrophysiologic Role

  2016cites this paper
• Fatty Acid Regulation of Voltage- and Ligand-Gated Ion Channel Function

  2016cites this paper
• Physiological and Pathophysiological Insights of Nav1.4 and Nav1.5 Comparison

  2016cites this paper
• Novel Clinical Manifestation of the Known SCN5A D1790G Mutation

  2015cites this paper
• On the multiple roles of the voltage gated sodium channel β1 subunit in genetic diseases

  2015cites this paper
• The genetic basis for inherited forms of sinoatrial dysfunction and atrioventricular node dysfunction

  2015cites this paper
• Absence of Family History and Phenotype–Genotype Correlation in Pediatric Brugada Syndrome: More Burden to Bear in Clinical and Genetic Diagnosis

  2015cites this paper
• NaV1.5 and Regulatory β Subunits in Cardiac Sodium Channelopathies

  2014cites this paper
• Channelopathies and Heart Disease

  2014cites this paper
• Antisense‐mediated post‐transcriptional silencing of SCN1B gene modulates sodium channel functional expression

  2014cites this paper
• Cardiac sodium channel mutations: why so many phenotypes?

  2014cites this paper
• Role of voltage-gated sodium channel beta subunit signaling in neuronal development and cancer

  2013cites this paper
• A biphasic role for the voltage-gated sodium channel scn5Lab in cardiac development of zebrafish

  2013cites this paper
• The Genetics of Cardiac Electrophysiology in Humans

  2013cites this paper
• Voltage-gated sodium channel-associated proteins and alternative mechanisms of inactivation and block

  2012cites this paper
• PERTURBATION OF SODIUM CHANNEL STRUCTURE BY AN INHERITED LONG QT SYNDROME MUTATION

  2012cites this paper
• Genetics and Cardiac Arrhythmia Syndromes

  2012cites this paper
• A hot topic

  2012cites this paper
• Mouse Models of SCN5A-Related Cardiac Arrhythmias

  2012cites this paper
• Human Nav1.5 F1486 deletion associated with long-QT syndrome leads to deficiency in inactivation and reduces lidocaine sensitivity

  2011cites this paper
• Solution NMR structure of Apo-calmodulin in complex with the IQ motif of human cardiac sodium channel NaV1.5.

  2011cites this paper
• Phenotypical Manifestations of Mutations in the Genes Encoding Subunits of the Cardiac Sodium Channel

  2011cites this paper
• Molecular Mechanisms of Voltage-Gated Na+ Channel Dysfunction in LQT3 Syndrome

  2011cites this paper
• Fibroblast growth factor homologous factors in the heart: a potential locus for cardiac arrhythmias.

  2011cites this paper
• Genetics and Genomics of Arrhythmias

  2010cites this paper
• Sodium Channel Carboxyl-terminal Residue Regulates Fast Inactivation*

  2010cites this paper
• Mutation-specific risk in two genetic forms of type 3 long QT syndrome.

  2010cites this paper
• Biophysical Characterization of Three SCN5A Mutations Linked to Long QT Syndrome Type 3, Sudden Infant Death Syndrome, and Atrial Fibrillation

  2010cites this paper
• Risk Stratification in Young Patients With Channelopathies

  2010cites this paper
• Autonomic modulation and antiarrhythmic therapy in a model of long QT syndrome type 3

  2010cites this paper
• Electrophysiological Characterization of Sodium Currents in Adult Rat Cardiac Myocytes

  2010cites this paper
• Novel mutation of SCN1A in familial generalized epilepsy with febrile seizures plus.

  2010cites this paper
• Genetic analysis of Brugada syndrome and congenital long-QT syndrome type 3 in the Chinese

  2010cites this paper
• Late Na+ current produced by human cardiac Na+ channel isoform Nav1.5 is modulated by its β1 subunit

  2009cites this paper
• A novel adhesion molecule in human breast cancer cells: voltage-gated Na+ channel beta1 subunit.

  2009cites this paper
• From Fifth Business to Protagonist: the complex roles of ion channel anchors in cardiac arrhythmia.

  2009cites this paper
• Solution NMR Structure of the C-terminal EF-hand Domain of Human Cardiac Sodium Channel NaV1.5*

  2009cites this paper
• Role of N- and C- termini in inactivation of sodium channel in weakly electric fish

  2009cites this paper
• Solution Structure of the NaV1.2 C-terminal EF-hand Domain*

  2009cites this paper
• The genetic basis of long QT and short QT syndromes: A mutation update

  2009cites this paper
• A Mutation in the β3 Subunit of the Cardiac Sodium Channel Associated With Brugada ECG Phenotype

  2009cites this paper
• CHAPTER 62 – Genetics and Genomics of Arrhythmias

  2009cites this paper
• Calcium-Mediated Dual-Mode Regulation of Cardiac Sodium Channel Gating

  2009cites this paper
• Functional characterization of voltage-gated sodium channels associated with human idiopathic epilepsies

  2009cites this paper
• AMP: adenosine monophosphate ARVC: arrhythmogenic right ventricular cardiomyopathy BrS: Brugada syndrome CAMs: cell adhesion molecules cAMP: cyclic adenosine monophosphate CaMKII: calcium/calmodulin dependent protein kinase II Cyto-D: cytochalasin-D DADs: delayed afterdepolarizations DGC: dystrophin

  2008cites this paper
• Sodium channel dysfunction in inherited and acquired cardiac diseases

  2008cites this paper
• Late sodium current in failing heart: friend or foe?

  2008cites this paper
• Ion Channels in Medicine

  2008cites this paper
• Functional Characterization of SCN5A, the Cardiac Sodium Channel Gene Associated with Cardiac Arrhythmias and Sudden Death

  2008cites this paper
• A mutation in the sodium channel is responsible for the association of long QT syndrome and familial atrial fibrillation.

  2008cites this paper
• Autonomic modulation of ventricular repolarization in Long QT syndrome

  2008cites this paper
• Importance of Knowing the Genotype and the Specific Mutation When Managing Patients With Long-QT Syndrome

  2008cites this paper
• Molecular cloning and analysis of zebrafish voltage-gated sodium channel beta subunit genes: implications for the evolution of electrical signaling in vertebrates

  2007cites this paper
• Fibroblast growth factor homologous factors control neuronal excitability through modulation of voltage-gated sodium channels.

  2007cites this paper
• Sodium channel Scn1b null mice exhibit prolonged QT and RR intervals.

  2007cites this paper
• Protein-protein interactions and membrane localization of the human organic solute transporter.

  2007cites this paper
• Flecainide sensitivity of a Na channel long QT mutation shows an open-channel blocking mechanism for use-dependent block.

  2006cites this paper
• Molecular basis of ranolazine block of LQT‐3 mutant sodium channels: evidence for site of action

  2006cites this paper
• Mathematical Simulations of the Effects of Altered AMP‐Kinase Activity on INa and the Action Potential in Rat Ventricle

  2006cites this paper
• ATX‐II Effects on the Apparent Location of M Cells in a Computational Model of a Human Left Ventricular Wedge

  2006cites this paper
• A Carboxyl-terminal Hydrophobic Interface Is Critical to Sodium Channel Function

  2006cites this paper
• Calcium-dependent regulation of the voltage-gated sodium channel hH1: intrinsic and extrinsic sensors use a common molecular switch.

  2006cites this paper
• Gene-specific therapy for inherited arrhythmogenic diseases.

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

  2006cites this paper
• Mutation screening for the genes causing cardiac arrhythmias.

  2006cites this paper
• β1-subunit modulates the Nav1.4 sodium channel by changing the surface charge

  2006cites this paper
• Temporal and spatial expression pattern of beta1 sodium channel subunit during heart development.

  2005cites this paper
• Molecular physiology of cardiac repolarization.

  2005cites this paper
• The Antiarrhythmic Effect of n-3 Polyunsaturated Fatty Acids: Modulation of Cardiac Ion Channels as a Potential Mechanism

  2005cites this paper
• Common human SCN5A polymorphisms have altered electrophysiology when expressed in Q1077 splice variants.

  2005cites this paper
• Sodium channels as macromolecular complexes: implications for inherited arrhythmia syndromes.

  2005cites this paper
• New aspects of vulnerability in heterogeneous models of ventricular wall and its modulation by loss of cardiac sodium channel function

  2005cites this paper
• Inherited and acquired vulnerability to ventricular arrhythmias: cardiac Na+ and K+ channels.

  2005cites this paper
• Congenital and Acquired Long QT Syndrome: Current Concepts and Management

  2004cites this paper
• Post-transcriptional alterations in the expression of cardiac Na+ channel subunits in chronic heart failure.

  2004cites this paper
• Functional role of the C‐terminus of voltage‐gated sodium channel Nav1.8

  2004cites this paper
• Modulation of Nav1.5 by β1- and β3-subunit co-expression in mammalian cells

  2004cites this paper
• Brugada Syndrome – An Under-Recognized Electrical Disease in Patients with Sudden Cardiac Death

  2004cites this paper
• Molecular genetic basis of sudden cardiac death.

  2004cites this paper
• Genetics of Long QT, Brugada, and Other Channelopathies

  2004cites this paper
• A Novel Epilepsy Mutation in the Sodium Channel SCN1A Identifies a Cytoplasmic Domain for β Subunit Interaction

  2004cites this paper
• Structural effects of an LQT-3 mutation on heart Na+ channel gating.

  2004cites this paper
• The common SCN5A mutation R1193Q causes LQTS-type electrophysiological alterations of the cardiac sodium channel

  2004cites this paper