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Absence of Dystrophin Disrupts Skeletal Muscle Signaling: Roles of Ca2+, Reactive Oxygen Species, and Nitric Oxide in the Development of Muscular Dystrophy
Open accessAbstract
Dystrophin is a long rod-shaped protein that connects the subsarcolemmal cytoskeleton to a complex of proteins in the surface membrane (dystrophin protein complex, DPC), with further connections via laminin to other extracellular matrix proteins. Initially considered a structural complex that protected the sarcolemma from mechanical damage, the DPC is now known to serve as a scaffold for numerous signaling proteins. Absence or reduced expression of dystrophin or many of the DPC components cause the muscular dystrophies, a group of inherited diseases in which repeated bouts of muscle damage lead to atrophy and fibrosis, and eventually muscle degeneration. The normal function of dystrophin is poorly defined. In its absence a complex series of changes occur with multiple muscle proteins showing reduced or increased expression or being modified in various ways. In this review, we will consider the various proteins whose expression and function is changed in muscular dystrophies, focusing on Ca 2+-permeable channels, nitric oxide synthase, NADPH oxidase, and caveolins. Excessive Ca 2+entry, increased membrane permeability, disordered caveolar function, and increased levels of reactive oxygen species are early changes in the disease, and the hypotheses for these phenomena will be critically considered. The aim of the review is to define the early damage pathways in muscular dystrophy which might be appropriate targets for therapy designed to minimize the muscle degeneration and slow the progression of the disease.
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Brenman, J. E., Xia, H., Chao, D. S., Black, S. M., & Bredt, D. S. (1997) · Developmental Neuroscience · doi:10.1159/000111211 · Cited by 161
Desmuslin, an intermediate filament protein that interacts with α-dystrobrevin and desmin
Mizuno, Y., Thompson, T. G., Guyon, J. R., Lidov, H. G. W., Brosius, M., Imamura, M., Ozawa, E., Watkins, S. C., & Kunkel, L. M. (2001) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.111153298 · Cited by 160
Zatz, M., Rapaport, D., Vainzof, M., Passos-Bueno, M. R., Bortolini, E. R., Pavanello, R. D. C. M., & Peres, C. A. (1991) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(91)90068-i · Cited by 159
Cited by
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“, intracellular calcium homeostasis inflammation and ROS metabolism) have been implicated as early events in the pathophysiology of this diseases [68].”
BackgroundBeekman, C., Janson, A. A., Baghat, A., van Deutekom, J. C., & Datson, N. A. (2018) · PLOS ONE · doi:10.1371/journal.pone.0195850 · Cited by 119
Vignaud, J., Loiseau, C., Hérault, J., Mayer, C., Côme, M., Martin, I., & Ulmann, L. (2023) · Antioxidants · doi:10.3390/antiox12051050 · Cited by 72
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Metadata
- Authors
- Allen, D. G., Whitehead, N. P., & Froehner, S. C.
- Venue
- Physiological Reviews
- Publisher
- American Physiological Society
- Published
- 2016-01
- Volume
- 96
- Issue
- 1
- Pages
- 253-305
- ISSN
- 0031-9333 (ISSN-L)
- 1522-1210
- OpenAlex
- W2206766868
- Cited by
- 426
- References
- 494
- FWCI
- 2.02
- Type
- journal article
- Language
- en
- Open access
- green
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
Data sources
Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.