Mexiletine block of disease‐associated mutations in S6 segments of the human skeletal muscle Na+ channel

No abstract is available for this paper.

• Publication year

  2001

• Venue

  Journal of Physiology

• Publication date

  2001-12-01

• Fields of study

  Medicine, Chemistry

• Identifiers
  DOI 10.1111/j.1469-7793.2001.00701.xPMID 11744749PMCID PMC2278988
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

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  2018influential reference
• Mexiletine

  2012influential reference
• Improved therapy of myotonia with the lidocaine derivative tocainide

  2004cited by this paper
• Residues in Na(+) channel D3-S6 segment modulate both batrachotoxin and local anesthetic affinities.

  2000cited by this paper
• Different effects of mexiletine on two mutant sodium channels causing paramyotonia congenita and hyperkalemic periodic paralysis.

  2000cited by this paper
• Defective slow inactivation of sodium channels contributes to familial periodic paralysis

  1999cited by this paper
• Enhanced slow inactivation by V445M: a sodium channel mutation associated with myotonia.

  1999cited by this paper
• The Position of the Fast-Inactivation Gate during Lidocaine Block of Voltage-gated Na+ Channels

  1999cited by this paper
• Voltage-gated ion channels and hereditary disease.

  1999cited by this paper
• Functional expression of the Ile693Thr Na+ channel mutation associated with paramyotonia congenita in a human cell line

  1998cited by this paper
• Effects of local anesthetics on Na+ channels containing the equine hyperkalemic periodic paralysis mutation.

  1998influential reference
• Effects of local anesthetics on Na+ channels containing the equine hyperkalemic periodic paralysis mutation.

  1998cited by this paper
• Human sodium channel gating defects caused by missense mutations in S6 segments associated with myotonia: S804F and V1293I

  1998cited by this paper
• Local anesthetic block of batrachotoxin-resistant muscle Na+ channels.

  1998cited by this paper
• Slow inactivation in human cardiac sodium channels.

  1998cited by this paper
• Effects of temperature and mexiletine on the F1473S Na+ channel mutation causing paramyotonia congenita

  1998cited by this paper
• A novel muscle sodium channel mutation causes painful congenital myotonia

  1997cited by this paper
• Sodium channel selectivity filter regulates antiarrhythmic drug binding.

  1997cited by this paper
• A common local anesthetic receptor for benzocaine and etidocaine in voltage-gated μ1 Na+ channels

  1997cited by this paper
• Evaluation of the antimyotonic activity of mexiletine and some new analogs on sodium currents of single muscle fibers and on the abnormal excitability of the myotonic ADR mouse.

  1997cited by this paper
• Differences in steady-state inactivation between Na channel isoforms affect local anesthetic binding affinity.

  1997cited by this paper
• Slow inactivation differs among mutant Na channels associated with myotonia and periodic paralysis.

  1997cited by this paper
• From mutation to myotonia in sodium channel disorders.

  1997cited by this paper
• Two human paramyotonia congenita mutations have opposite effects on lidocaine block of Na+ channels expressed in a mammalian cell line.

  1996influential reference
• Inactivation defects caused by myotonia-associated mutations in the sodium channel III-IV linker

  1996cited by this paper
• The skeletal muscle sodium and chloride channel diseases.

  1995cited by this paper
• Different effects on gating of three myotonia‐causing mutations in the inactivation gate of the human muscle sodium channel.

  1995cited by this paper
• Paramyotonia congenita: Abnormal short exercise test, and improvement after mexiletine therapy

  1994cited by this paper
• K(+)‐aggravated myotonia: destabilization of the inactivated state of the human muscle Na+ channel by the V1589M mutation.

  1994cited by this paper
• Visual identification of individual transfected cells for electrophysiology using antibody-coated beads.

  1994cited by this paper
• Molecular determinants of state-dependent block of Na+ channels by local anesthetics.

  1994cited by this paper
• Functional consequences of a Na+ channel mutation causing hyperkalemic periodic paralysis.

  1993cited by this paper
• The cloning and expression of a sodium channel beta 1-subunit cDNA from human brain.

  1993cited by this paper
• Theoretical reconstruction of myotonia and paralysis caused by incomplete inactivation of sodium channels.

  1993cited by this paper
• The cloning and expression of a sodium channel β1-subunit cDNA from human brain

  1993cited by this paper
• Primary structure of the adult human skeletal muscle voltage‐dependent sodium channel

  1992cited by this paper
• Molecular Mechanisms of Local Anesthesia: A Review

  1990influential reference
• Acetazolamide‐responsive myotonia congenita

  1987cited by this paper
• Lidocaine block of cardiac sodium channels

  1983cited by this paper
• Local anesthetics: hydrophilic and hydrophobic pathways for the drug- receptor reaction

  1977cited by this paper
• The ionic channels in excitable membranes.

  1975influential reference

• Evaluation of professional practices in the use of mexiletine for the management of childhood myotonia in French pediatric neuromuscular centers (MEXI-PEDI survey).

  2025cites this paper
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  2024cites this paper
• Molecular mechanisms and therapeutic strategies for neuromuscular diseases

  2024cites this paper
• Effectiveness and safety of mexiletine versus placebo in patients with myotonia: a systematic review and meta-analysis

  2024cites this paper
• Hyperkalemic periodic paralysis associated with a novel missense variant located in the inner pore of Nav1.4.

  2023cites this paper
• The myotonic dystrophy type 1 drug development pipeline: 2022 edition.

  2023cites this paper
• Beneficial effects of chronic mexiletine treatment in a human model of SCN5A overlap syndrome

  2023cites this paper
• Erythromycin for myotonic dystrophy type 1: a multicentre, randomised, double-blind, placebo-controlled, phase 2 trial

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  2020cites this paper
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  2020cites this paper
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  2020cites this paper
• Chronic inflammatory demyelinating polyradiculoneuropathy relapse after mexiletine withdrawal in a patient with concomitant myotonia congenita

  2020cites this paper
• Pharmacogenetics of myotonic hNav1.4 sodium channel variants situated near the fast inactivation gate

  2019cites this paper
• Sodium Channelopathies: From Molecular Physiology towards Medical Genetics

  2018cites this paper
• Therapeutic Approaches to Genetic Ion Channelopathies and Perspectives in Drug Discovery

  2016cites this paper
• Translational approach to address therapy in myotonia permanens due to a new SCN4A mutation

  2016cites this paper
• Mechanism of Modification, by Lidocaine, of Fast and Slow Recovery from Inactivation of Voltage-Gated Na+ Channels

  2015cites this paper
• Mexiletine for treatment of myotonia: a trial triumph for rare disease networks.

  2012cites this paper
• Biophysical characterization of neuronal and skeletal muscle sodium channels, and their regulation by auxiliary beta subunits

  2012cites this paper
• The Na conductance in the sarcolemma and the transverse tubular system membranes of mammalian skeletal muscle fibers

  2011cites this paper
• Skeletal Muscle Na+ Channel Disorders

  2011cites this paper
• Caractérisation clinique et génétique des myotonies congénitales classiques et atypiques au Saguenay Lac St-Jean

  2010cites this paper
• Comparison of the effect of mexiletine and methylprednisolone on neural function and histopathological damage after transient spinal cord ischemia in rabbits.

  2010cites this paper
• Mexiletine is an effective antimyotonia treatment in myotonic dystrophy type 1

  2010cites this paper
• Disorders of Voluntary Muscle: Dystrophinopathies

  2010cites this paper
• A novel Nav1.7 mutation producing carbamazepine‐responsive erythromelalgia

  2009cites this paper
• Therapeutic approaches to ion channel diseases.

  2008cites this paper
• Treatment of neuromuscular channelopathies: Current concepts and future prospects

  2008cites this paper
• Bupivacaine blocks N-type inactivating Kv channels in the open state: no allosteric effect on inactivation kinetics.

  2008cites this paper
• Canalopatías autoinmunes del sistema nervioso central

  2007cites this paper
• Effects of chronic sepsis on the voltage-gated sodium channel in isolated rat muscle fibers*

  2007cites this paper
• Ion channel pharmacology

  2007cites this paper
• Speeding the recovery from ultraslow inactivation of voltage-gated Na+ channels by metal ion binding to the selectivity filter: a foot-on-the-door?

  2007cites this paper
• Outward stabilization of the S4 segments in domains III and IV enhances lidocaine block of sodium channels

  2007influential citation
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  2006cites this paper
• Inherited disorders of voltage-gated sodium channels.

  2005cites this paper
• Preferred mexiletine block of human sodium channels with IVS4 mutations and its pH-dependence*

  2005cites this paper
• Sodium channel inactivation: molecular determinants and modulation.

  2005cites this paper
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  2005cites this paper
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