ArticleThe Journal of cell biology1999
Extensive but coordinated reorganization of the membrane skeleton in myofibers of dystrophic (mdx) mice.
Article in The Journal of cell biology, 1999. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers.
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Who cites it
37 citing papers in PubMed, 93 citations in OpenAlex.
- Review
- Dystrophin missense mutations alter focal adhesion tension and mechanotransduction.Proceedings of the National Academy of Sciences of the United States of America · 2022Article
- The Donnan-dominated resting state of skeletal muscle fibers contributes to resilience and longevity in dystrophic fibers.The Journal of general physiology · 2022Article
- Microutrophin expression in dystrophic mice displays myofiber type differences in therapeutic effects.PLoS genetics · 2020Article
- Dystrophin As a Molecular Shock Absorber.ACS nano · 2018Article
- Multiscale models of skeletal muscle reveal the complex effects of muscular dystrophy on tissue mechanics and damage susceptibility.Interface focus · 2015Article
- Filamentous structures in skeletal muscle: anchors for the subsarcolemmal space.Medical molecular morphology · 2015Article
- Utrophin suppresses low frequency oscillations and coupled gating of mechanosensitive ion channels in dystrophic skeletal muscle.Channels (Austin, Tex.) · 2015Article
- Utrophin regulates modal gating of mechanosensitive ion channels in dystrophic skeletal muscle.The Journal of physiology · 2014Article
- Dystrophin deficiency leads to disturbance of LAMP1-vesicle-associated protein secretion.Cellular and molecular life sciences : CMLS · 2013Article
- Obscurin is required for ankyrinB-dependent dystrophin localization and sarcolemma integrity.The Journal of cell biology · 2013Article
- Interaction of α-catulin with dystrobrevin contributes to integrity of dystrophin complex in muscle.The Journal of biological chemistry · 2012Article
- The passive mechanical properties of the extensor digitorum longus muscle are compromised in 2- to 20-mo-old mdx mice.Journal of applied physiology (Bethesda, Md. : 1985) · 2011Article
- Biomechanics of the sarcolemma and costameres in single skeletal muscle fibers from normal and dystrophin-null mice.Journal of muscle research and cell motility · 2011Article
- Characterization and expression of a heart-selective alternatively spliced variant of alpha II-spectrin, cardi+, during development in the rat.Journal of molecular and cellular cardiology · 2010Article
- Cell membrane expression of cardiac sodium channel Na(v)1.5 is modulated by alpha-actinin-2 interaction.Biochemistry · 2010Article
- Obscurin interacts with a novel isoform of MyBP-C slow at the periphery of the sarcomeric M-band and regulates thick filament assembly.Molecular biology of the cell · 2009Article
- Applications of metabolomics and proteomics to the mdx mouse model of Duchenne muscular dystrophy: lessons from downstream of the transcriptome.Genome medicine · 2009Article
- Nav1.4 deregulation in dystrophic skeletal muscle leads to Na+ overload and enhanced cell death.The Journal of general physiology · 2008Article
- Destabilization of the dystrophin-glycoprotein complex without functional deficits in alpha-dystrobrevin null muscle.PloS one · 2008Article
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
We used immunofluorescence techniques and confocal imaging to study the organization of the membrane skeleton of skeletal muscle fibers of mdx mice, which lack dystrophin. beta-Spectrin is normally found at the sarcolemma in costameres, a rectilinear array of longitudinal strands and elements overlying Z and M lines. However, in the skeletal muscle of mdx mice, beta-spectrin tends to be absent from the sarcolemma over M lines and the longitudinal strands may be disrupted or missing. Other proteins of the membrane and associated cytoskeleton, including syntrophin, beta-dystroglycan, vinculin, and Na,K-ATPase are also concentrated in costameres, in control myofibers, and mdx muscle. They also distribute into the same altered sarcolemmal arrays that contain beta-spectrin. Utrophin, which is expressed in mdx muscle, also codistributes with beta-spectrin at the mutant sarcolemma. By contrast, the distribution of structural and intracellular membrane proteins, including alpha-actinin, the Ca-ATPase and dihydropyridine receptors, is not affected, even at sites close to the sarcolemma. Our results suggest that in myofibers of the mdx mouse, the membrane- associated cytoskeleton, but not the nearby myoplasm, undergoes widespread coordinated changes in organization. These changes may contribute to the fragility of the sarcolemma of dystrophic muscle.
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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.