Rippling muscle disease may be caused by “silent” action potentials in the tubular system of skeletal muscle fibers
Abstract
Rippling muscle disease (RMD) is a generally benign, myotonic‐like myopathy associated with rapid rolling contractions and percussion‐induced contractions. These contractions are electrically silent in electromyographic recordings, which is taken as evidence that action potentials are not involved in the phenomena. The pathophysiological mechanisms underlying the symptoms have not been elucidated. Many cases of RMD are caused by mutations in caveolin‐3, and aberrations in the tubular system are commonly observed. Here, recent data are discussed showing that action potentials can travel over substantial distances entirely within the transverse and longitudinal tubular systems of a muscle fiber and that stretch can induce such action potentials. Action potentials travelling in the tubular system in most circustances probably cannot excite the sarcolemma and hence would not be dected. It is suggested that the distinctive contractions in RMD may be due to stretch‐induced generation of action potentials within the tubular system. Muscle Nerve, 2005
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- Authors
- Lamb, G. D.
- Affiliations
- La Trobe University (AU)
- Corresponding author
- Lamb, Graham D.
- ORCID
- Lamb, Graham D.
- Venue
- Muscle & Nerve
- Publisher
- Wiley
- Published
- 2005-03
- Volume
- 31
- Issue
- 5
- Pages
- 652-658
- ISSN
- 0148-639X (ISSN-L)
- 1097-4598
- OpenAlex
- W2077324939
- Cited by
- 47
- References
- 42
- Citations by year
- 2026: 2 · 2025: 3 · 2024: 3 · 2022: 1 · 2021: 3 · 2020: 2 · 2017: 1 · 2015: 1 · 2014: 3 · 2013: 3 · 2012: 2
- FWCI
- 0.32
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Muscle & Nerve (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Muscle Physiology and Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ion channel regulation and function
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Caveolin-1 and cellular processes
- Keywords
- rippling muscle disease, transverse tubular system, caveolin-3, muscle fiber excitation, sarcolemma
- MeSH terms
- Action Potentials, Animals, Cell Membrane, Humans, Muscle Contraction, Muscular Diseases, Mutation, Reflex, Stretch, Stress, Mechanical, Muscle, Skeletal, Muscle Fibers, Skeletal, Caveolins, Caveolin 3, Rats
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