ArticleThe Journal of physiology1997
Human Na+ channel fast and slow inactivation in paramyotonia congenita mutants expressed in Xenopus laevis oocytes.
Article in The Journal of physiology, 1997. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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21 citing papers in PubMed, 46 citations in OpenAlex.
- Characterization of a new sodium channel mutation at arginine 1448 associated with moderate Paramyotonia congenita in humans.The Journal of physiology · 1999Trial
- Clinical, electromyographic, and biophysical characterization of the rare Nav1.4 channel mutationFrontiers in physiology · 2025Article
- Article
- Changes in Resurgent Sodium Current Contribute to the Hyperexcitability of Muscles in Patients with Paramyotonia Congenita.Biomedicines · 2021Article
- NaScientific reports · 2018Article
- Mechanisms of a human skeletal myotonia produced by mutation in the C-terminus of NaV1.4: is Ca2+ regulation defective?PloS one · 2013Article
- Skeletal muscle na channel disorders.Frontiers in pharmacology · 2011Article
- An epilepsy mutation in the sodium channel SCN1A that decreases channel excitability.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2006Article
- Functional characterization and cold sensitivity of T1313A, a new mutation of the skeletal muscle sodium channel causing paramyotonia congenita in humans.The Journal of physiology · 2004Article
- Interaction of scorpion alpha-toxins with cardiac sodium channels: binding properties and enhancement of slow inactivation.The Journal of general physiology · 2001Article
- A missense mutation of the Na+ channel alpha II subunit gene Na(v)1.2 in a patient with febrile and afebrile seizures causes channel dysfunction.Proceedings of the National Academy of Sciences of the United States of America · 2001Article
- Myopathic mutations affect differently the inactivation of the two gating modes of sodium channels.Journal of bioenergetics and biomembranes · 1999Article
- Enhanced slow inactivation by V445M: a sodium channel mutation associated with myotonia.Biophysical journal · 1999Article
- Fast- and slow-gating modes of the sodium channel are altered by a paramyotonia congenita-linked mutation.Journal of bioenergetics and biomembranes · 1998Article
- Human sodium channel gating defects caused by missense mutations in S6 segments associated with myotonia: S804F and V1293I.The Journal of physiology · 1998Article
- Slow inactivation in human cardiac sodium channels.Biophysical journal · 1998Article
- Functional expression of the Ile693Thr Na+ channel mutation associated with paramyotonia congenita in a human cell line.The Journal of physiology · 1998Article
- A defect in skeletal muscle sodium channel deactivation exacerbates hyperexcitability in human paramyotonia congenita.The Journal of physiology · 1998Article
- Voltage-induced slow activation and deactivation of mechanosensitive channels in Xenopus oocytes.The Journal of physiology · 1997Article
- Defective fast inactivation recovery and deactivation account for sodium channel myotonia in the I1160V mutant.Biophysical journal · 1997Article
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3 authors at 1 institution in 1 country.
Funding
Abstract
1. Paramyotonia congenita (PC) is a human hereditary disease caused by one or more amino acid substitutions in skeletal muscle sodium channels. Using macropatches, the effect of PC mutations R1448C and T1313M were compared with wild-type (WT) in Xenopus oocytes coinjected with both alpha- and beta-subunits of human skeletal muscle (SkM1) sodium channels. 2. Slow inactivation in either T1313M or R1448C was not different from WT. Fast inactivation in both PC mutants, however, was significantly altered. 3. Commonly used biophysical protocols (such as I-V curves, steady-state inactivation curves, and measurements of inactivation rates) did not uniformly indicate that hyperexcitability should result from T1313M or R1448C. In fact, the only alteration of fast inactivation common to T1313M and R1448C that predicted cellular hyperexcitability was slowed open-state inactivation, compared with WT. 4. To test whether this alteration was sufficient to cause the phenotypic hyperexcitability, we used a novel voltage command that simulated muscle membrane activity. With this protocol, we found that R1448C and T1313M were similar in that they maintained a significantly higher channel availability during high frequency activity, compared with WT.
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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.