First Author | Settles DL | Year | 1996 |
Journal | Muscle Nerve | Volume | 19 |
Issue | 2 | Pages | 147-54 |
PubMed ID | 8559162 | Mgi Jnum | J:31694 |
Mgi Id | MGI:79181 | Doi | 10.1002/(SICI)1097-4598(199602)19:2<147::AID-MUS4>3.0.CO;2-E |
Citation | Settles DL, et al. (1996) Tenascin-C expression in dystrophin-related muscular dystrophy. Muscle Nerve 19(2):147-54 |
abstractText | 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. |