Tenascin-C expression in dystrophin-related muscular dystrophy.
The mdx mouse has a mutated dystrophin gene and is used as a model for the study of Duchenne muscular dystrophy (DMD). We investigated whether regenerating mdx skeletal muscle contains the extracellular matrix protein tenascin-C (TN-C), which is expressed in wound healing and nerve regeneration. Prior to the initiation of muscle degeneration, both normal and mdx mice displayed similar weak staining for TN-C in skeletal muscle, but by 3 weeks of age the mice differed substantially. TN-C was undetectable in normal muscle except at the myotendinous junction, while in dystrophic muscle, TN-C was prominent in degenerating/regenerating areas, but absent from undegenerated muscle. With increasing age, TN-C staining declined around stable regenerated mdx myofibers. TN-C was also observed in muscle from dogs with muscular dystrophy and in human boys with DMD. Therefore, in dystrophic muscle, TN-C expression may be stimulated by the degenerative process and remain upregulated unless the tissue undergoes successful regeneration.
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Related Subject Headings
- Wound Healing
- Tenascin
- Neurology & Neurosurgery
- Nerve Regeneration
- Muscular Dystrophy, Animal
- Mice, Inbred C57BL
- Mice
- Immunohistochemistry
- Humans
- Dystrophin
Citation
Published In
DOI
ISSN
Publication Date
Volume
Issue
Start / End Page
Location
Related Subject Headings
- Wound Healing
- Tenascin
- Neurology & Neurosurgery
- Nerve Regeneration
- Muscular Dystrophy, Animal
- Mice, Inbred C57BL
- Mice
- Immunohistochemistry
- Humans
- Dystrophin