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MiRP2 Forms Potassium Channels in Skeletal Muscle with Kv3.4 and Is Associated with Periodic Paralysis
Open accessAbstract
The subthreshold, voltage-gated potassium channel of skeletal muscle is shown to contain MinK-related peptide 2 (MiRP2) and the pore-forming subunit Kv3.4. MiRP2-Kv3.4 channels differ from Kv3.4 channels in unitary conductance, voltage-dependent activation, recovery from inactivation, steady-state open probability, and block by a peptide toxin. Thus, MiRP2-Kv3.4 channels set resting membrane potential (RMP) and do not produce afterhyperpolarization or cumulative inactivation to limit action potential frequency. A missense mutation is identified in the gene for MiRP2 (KCNE3) in two families with periodic paralysis and found to segregate with the disease. Mutant MiRP2-Kv3.4 complexes exhibit reduced current density and diminished capacity to set RMP. Thus, MiRP2 operates with a classical potassium channel subunit to govern skeletal muscle function and pathophysiology.
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“The MiRP2-Kv3.4 mutation reduces potassium efflux, resulting in hypokalemia. This condition decreases muscle membrane excitability, leading to muscle weakness and paralysis [19].”
From Thyrotoxic Hypokalemic Periodic Paralysis: Pathophysiological Mechanisms (2025) · cites this paper as [19]
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Metadata
- Authors
- Abbott, G. W., Butler, M. H., Bendahhou, S., Dalakas, M. C., Ptacek, L. J., & Goldstein, S. A. N.
- Affiliations
- Yale University (US)
- National Institutes of Health (US)
- Corresponding author
- Goldstein, Steve A.N
- Venue
- Cell
- Publisher
- Elsevier BV
- Published
- 2001-01
- Volume
- 104
- Issue
- 2
- Pages
- 217-231
- ISSN
- 0092-8674 (ISSN-L)
- OpenAlex
- W2069886131
- Cited by
- 294
- References
- 57
- Citations by year
- 2026: 7 · 2025: 8 · 2024: 2 · 2023: 2 · 2022: 3 · 2021: 5 · 2020: 4 · 2019: 6 · 2018: 4 · 2017: 4 · 2016: 7 · 2015: 5 · 2014: 9 · 2013: 11 · 2012: 5
- FWCI
- 11.64
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Cell (publishedVersion)
- PubMed (publishedVersion)
- eScholarship (California Digital Library) (submittedVersion, cc-by)
- Funders
- National Center for Research Resources (MO1-RR-00064, M01RR000064)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ion channel regulation and function
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ion Transport and Channel Regulation
- Keywords
- KCNE3, Kv3.4, voltage-gated potassium channels, skeletal muscle, periodic paralysis, resting membrane potential, single-channel conductance, channel inactivation
- MeSH terms
- Animals, Cell Line, Cell Membrane, Chromosome Mapping, Chromosomes, Human, Pair 11, Cnidarian Venoms, Electrophysiology, Female, Cricetinae, Humans, Immunohistochemistry, Male, Membrane Potentials, Oocytes, Paralyses, Familial Periodic, Pedigree, Xenopus laevis, Potassium Channels, Patch-Clamp Techniques, Muscle, Skeletal, Mutation, Missense, Protein Subunits, Potassium Channels, Voltage-Gated, Xenopus Proteins, Mice, Rats, Shaw Potassium Channels
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