Evidence map›Paper›PMID 40657735›Full record

ArticleAnnals of clinical and translational neurology2025

Discovery and Treatment of Action Potential-Independent Myotonia in Hyperkalemic Periodic Paralysis.

Chris Dupont, Adam Deardorff, Murad Nawaz, Andrew A Voss, Mark M Rich

Abstract read
In one paragraph

Article in Annals of clinical and translational neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

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0cells of the map it votes in
2citing papers in PubMed
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1 · What the graph read from it

What it found

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2 · The registry

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3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

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4 · The record

Corrections and comments

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5 · Who and what money

Authors and funding

5 authors.

Chris DupontDepartment of Neuroscience, Cell Biology and Physiology, Wright State University, Dayton, Ohio, USA.
Adam DeardorffDepartment of Neuroscience, Cell Biology and Physiology, Wright State University, Dayton, Ohio, USA.
Murad NawazDepartment of Neuroscience, Cell Biology and Physiology, Wright State University, Dayton, Ohio, USA.
Andrew A VossDepartment of Biological Sciences, Wright State University, Dayton, Ohio, USA.
Mark M RichDepartment of Neuroscience, Cell Biology and Physiology, Wright State University, Dayton, Ohio, USA.ORCID 0000-0002-6956-5531

Funding

Novel Approaches to Therapy of Muscle Ion ChannelopathiesR01AR074985 · NIAMS · WRIGHT STATE UNIVERSITY · PI MARK M RICH · 2019 to 2026
$3.9M
Treating Hyperkalemic Periodic ParalysisF30AR081675 · NIAMS · WRIGHT STATE UNIVERSITY · PI DUPONT, CHRISTOPHER · 2022 to 2025
$198k
NIAMS NIH HHS AR074985NIAMS NIH HHS AR081675NIAMS NIH HHS F30 AR081675NIAMS NIH HHS R01 AR074985
6 · The paper itself

Abstract

objectiveHyperkalemic periodic paralysis (hyperKPP) is characterized by attacks of transient weakness. A subset of hyperKPP patients suffers from transient involuntary contraction of muscle (myotonia). The goal of this study was to determine mechanisms causing myotonia in hyperKPP.

methodsIntracellular electrophysiology, single-fiber Ca

resultsMyotonia in hyperkPP was caused by both involuntary myogenic action potentials (AP myotonia) lasting less than 5 min and action potential-independent myotonia (non-AP myotonia) lasting over 1 h. Non-AP myotonia was caused by prolonged subthreshold depolarization and elevated intracellular Ca DISCUSSION: We propose myotonia in hyperKPP occurs via two mechanisms: (1) suprathreshold depolarization triggering action potentials that are detectable with EMG and (2) sustained subthreshold depolarization resulting in Na

Indexed as

Action PotentialsDantroleneMyotoniaParalysis, Hyperkalemic PeriodicAnimalsCalciumDisease Models, AnimalMaleMiceMuscle ContractionMuscle Relaxants, CentralMuscle, SkeletalCalciumDantroleneMuscle Relaxants, Centralaction potentialexcitationskeletal musclesodium channelstiffness

Identifiers

PMID40657735
PMCPMC12516254

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.