Tenascin-C expression in dystrophin-related muscular dystrophy

✍ Scribed by Daniel L. Settles; Robert A. Cihak; Harold P. Erickson

• Publisher: John Wiley and Sons
• Year: 1996
• Tongue: English
• Weight: 857 KB
• Volume: 19
• Category: Article
• ISSN: 0148-639X
• DOI: 10.1002/(sici)1097-4598(199602)19:2<147::aid-mus4>3.0.co;2-e

✦ Synopsis

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 degeneratinghegenerating areas, but absent from undegenerated muscle. With increasing age, TN-C staining declined around stable regenerated rndx 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.