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Defective membrane repair in dysferlin-deficient muscular dystrophy
Abstract
Muscular dystrophy includes a diverse group of inherited muscle diseases characterized by wasting and weakness of skeletal muscle. Mutations in dysferlin are linked to two clinically distinct muscle diseases, limb-girdle muscular dystrophy type 2B and Miyoshi myopathy, but the mechanism that leads to muscle degeneration is unknown. Dysferlin is a homologue of the Caenorhabditis elegans fer-1 gene, which mediates vesicle fusion to the plasma membrane in spermatids. Here we show that dysferlin-null mice maintain a functional dystrophin-glycoprotein complex but nevertheless develop a progressive muscular dystrophy. In normal muscle, membrane patches enriched in dysferlin can be detected in response to sarcolemma injuries. In contrast, there are sub-sarcolemmal accumulations of vesicles in dysferlin-null muscle. Membrane repair assays with a two-photon laser-scanning microscope demonstrated that wild-type muscle fibres efficiently reseal their sarcolemma in the presence of Ca2+. Interestingly, dysferlin-deficient muscle fibres are defective in Ca2+-dependent sarcolemma resealing. Membrane repair is therefore an active process in skeletal muscle fibres, and dysferlin has an essential role in this process. Our findings show that disruption of the muscle membrane repair machinery is responsible for dysferlin-deficient muscle degeneration, and highlight the importance of this basic cellular mechanism of membrane resealing in human disease.
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“DYSF is implicated in membrane repair22; ZNF638 mediates the silencing of unintegrated viral DNA23.”
From Locus for severity implicates CNS resilience in progression of multiple sclerosis (2023) · cites this paper as [22]
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“Although widely expressed, its functions are mainly characterized in skeletal muscle where it participates in calcium-mediated membrane repair and regeneration22.”
From Locus for severity implicates CNS resilience in progression of multiple sclerosis (2023) · cites this paper as [22]
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Metadata
- Authors
- Bansal, D., Miyake, K., Vogel, S. S., Groh, S., Chen, C.-C., Williamson, R., McNeil, P. L., & Campbell, K. P.
- Affiliations
- University of Iowa (US)
- Augusta University (US)
- National Institutes of Health (US)
- Corresponding author
- Campbell, Kevin P.
- Venue
- Nature
- Published
- 2003-05
- Volume
- 423
- Issue
- 6936
- Pages
- 168-172
- ISSN
- 0028-0836 (ISSN-L)
- 1476-4687
- OpenAlex
- W2047136624
- Cited by
- 1,022
- References
- 27
- Citations by year
- 2026: 22 · 2025: 35 · 2024: 47 · 2023: 35 · 2022: 53 · 2021: 42 · 2020: 64 · 2019: 41 · 2018: 36 · 2017: 38 · 2016: 56 · 2015: 55 · 2014: 55 · 2013: 48 · 2012: 51
- FWCI
- 12.30
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Nature (publishedVersion)
- PubMed (publishedVersion)
- RePEc: Research Papers in Economics (submittedVersion)
- Funders
- Howard Hughes Medical Institute
- National Aeronautics and Space Administration
- Muscular Dystrophy Association
- National Institutes of Health
- Roy J. and Lucille A. Carver College of Medicine, University of Iowa
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Muscle Physiology and Disorders
- Health Sciences › Medicine › Surgery › Muscle and Compartmental Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Cellular transport and secretion
- Keywords
- dysferlin, limb-girdle muscular dystrophy type 2B, Miyoshi myopathy, membrane repair, dystrophin-glycoprotein complex, muscle degeneration
- MeSH terms
- Dysferlin, Animals, Calcium, Cell Membrane, Membrane Fusion, Membrane Proteins, Muscle Proteins, Muscular Dystrophies, Regeneration, Stress, Mechanical, Dystrophin, Gene Deletion, Mice, Knockout, Muscle, Skeletal, Muscle Fibers, Skeletal, Mice
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