ArticleAnnals of neurology2017
Inhibiting persistent inward sodium currents prevents myotonia.
Article in Annals of neurology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers, 1 of them a synthesis that pooled it.
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Who cites it
19 citing papers in PubMed, 1 synthesis or guideline pooled it, 25 citations in OpenAlex.
- Targeted Therapies for Skeletal Muscle Ion Channelopathies: Systematic Review and Steps Towards Precision Medicine.Journal of neuromuscular diseases · 2021Pooled it
- Efficacy of sodium channel blockers in treating myotonia is independent of action potential sodium current block.The Journal of physiology · 2026Article
- Therapeutic Strategies Targeting the Molecular Pathogenesis of Myotonic Dystrophy Type 1: Current Status and Future Directions.Molecular diagnosis & therapy · 2026Review
- Discovery and Treatment of Action Potential-Independent Myotonia in Hyperkalemic Periodic Paralysis.Annals of clinical and translational neurology · 2025Article
- Mechanisms underlying the distinct K+ dependencies of periodic paralysis.The Journal of general physiology · 2025Article
- Reduced KThe Journal of physiology · 2024Article
- Preclinical study of the antimyotonic efficacy of safinamide in the myotonic mouse model.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2024Article
- Plateau potentials contribute to myotonia in mouse models of myotonia congenita.Experimental neurology · 2023Article
- A novel mutation of the CLCN1 gene in a cat with myotonia congenita: Diagnosis and treatment.Journal of veterinary internal medicine · 2022Article
- Ranolazine: An Old Drug with Emerging Potential; Lessons from Pre-Clinical and Clinical Investigations for Possible Repositioning.Pharmaceuticals (Basel, Switzerland) · 2021Review
- Article
- Preclinical pharmacological in vitro investigations on low chloride conductance myotonia: effects of potassium regulation.Pflugers Archiv : European journal of physiology · 2020Article
- Guidelines on clinical presentation and management of nondystrophic myotonias.Muscle & nerve · 2020Review
- TRPV4 Antagonism Prevents Mechanically Induced Myotonia.Annals of neurology · 2020Article
- Mechanisms of altered skeletal muscle action potentials in the R6/2 mouse model of Huntington's disease.American journal of physiology. Cell physiology · 2020Article
- Central Role of Subthreshold Currents in Myotonia.Annals of neurology · 2020Review
- Treatment Updates for Neuromuscular Channelopathies.Current treatment options in neurology · 2020Review
- Myotonic Myopathy With Secondary Joint and Skeletal Anomalies From the c.2386C>G, p.L769V Mutation inFrontiers in neurology · 2020Article
- Treatment of myotonia congenita with retigabine in mice.Experimental neurology · 2019Article
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
objectivePatients with myotonia congenita have muscle hyperexcitability due to loss-of-function mutations in the ClC-1 chloride channel in skeletal muscle, which causes involuntary firing of muscle action potentials (myotonia), producing muscle stiffness. The excitatory events that trigger myotonic action potentials in the absence of stabilizing ClC-1 current are not fully understood. Our goal was to identify currents that trigger spontaneous firing of muscle in the setting of reduced ClC-1 current.
methodsIn vitro intracellular current clamp and voltage clamp recordings were performed in muscle from a mouse model of myotonia congenita.
resultsIntracellular recordings revealed a slow afterdepolarization (AfD) that triggers myotonic action potentials. The AfD is well explained by a tetrodotoxin-sensitive and voltage-dependent Na
interpretationThis work significantly changes our understanding of the mechanisms triggering myotonia. Our work suggests that the current focus of treating myotonia, blocking the transient Na
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