ReviewThe Journal of physiology2010
Muscle channelopathies: does the predicted channel gating pore offer new treatment insights for hypokalaemic periodic paralysis?
Review in The Journal of physiology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
26 citing papers in PubMed, 60 citations in OpenAlex.
- Article
- Hypokalemic periodic paralysis in a teenage boy after an intense period of exercise: A rare case report.Clinical case reports · 2023Article
- Quantitative cerebrovascular reactivity MRI in mice using acetazolamide challenge.Magnetic resonance in medicine · 2022Article
- Recovery from acidosis is a robust trigger for loss of force in murine hypokalemic periodic paralysis.The Journal of general physiology · 2019Article
- Skeletal Muscle Channelopathies.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2018Review
- Evaluation of mutant muscle CaThe Journal of general physiology · 2018Article
- When muscle CaThe Journal of physiology · 2018Review
- Review of the Diagnosis and Treatment of Periodic Paralysis.Muscle & nerve · 2018Review
- Na leak with gating pore properties in hypokalemic periodic paralysis V876E mutant muscle Ca channel.The Journal of general physiology · 2017Article
- Elevated resting HThe Journal of physiology · 2017Article
- Phospholemman, a major regulator of skeletal muscle NaExperimental and therapeutic medicine · 2017Article
- Mice with an NaV1.4 sodium channel null allele have latent myasthenia, without susceptibility to periodic paralysis.Brain : a journal of neurology · 2016Article
- A novel NaV1.5 voltage sensor mutation associated with severe atrial and ventricular arrhythmias.Journal of molecular and cellular cardiology · 2016Article
- Review
- Therapeutic Approaches to Genetic Ion Channelopathies and Perspectives in Drug Discovery.Frontiers in pharmacology · 2016Review
- Channelopathies of skeletal muscle excitability.Comprehensive Physiology · 2015Review
- Beneficial effects of bumetanide in a CaV1.1-R528H mouse model of hypokalaemic periodic paralysis.Brain : a journal of neurology · 2013Article
- Autoantibodies to neurotransmitter receptors and ion channels: from neuromuscular to neuropsychiatric disorders.Frontiers in genetics · 2013Review
- Gating currents from Kv7 channels carrying neuronal hyperexcitability mutations in the voltage-sensing domain.Biophysical journal · 2012Article
- Pathophysiological role of omega pore current in channelopathies.Frontiers in pharmacology · 2012Article
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
Hypokalaemic periodic paralysis (hypoPP) is the archetypal skeletal muscle channelopathy caused by dysfunction of one of two sarcolemmal ion channels, either the sodium channel Nav1.4 or the calcium channel Cav1.1. Clinically, hypoPP is characterised by episodes of often severe flaccid muscle paralysis, in which the muscle fibre membrane becomes electrically inexcitable, and which may be precipitated by low serum potassium levels. Initial functional characterisation of hypoPP mutations failed to adequately explain the pathomechanism of the disease. Recently, as more pathogenic mutations involving loss of positive charge have been identified in the S4 segments of either channel, the hypothesis that an abnormal gating pore current may be important has emerged. Such an aberrant gating pore current has been identified in mutant Nav1.4 channels and has prompted potentially significant advances in this area. The carbonic anhydrase inhibitor acetazolamide has been used as a treatment for hypokalaemic periodic paralysis for over 40 years but its precise therapeutic mechanism of action is unclear. In this review we summarise the recent advances in the understanding of the molecular pathophysiology of hypoPP and consider how these may relate to the reported beneficial effects of acetazolamide. We also consider potential areas for future therapeutic development.
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Registered trials
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.