Tenascin-C expression in dystrophin-related muscular dystrophy.

Published

Journal Article

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.

Full Text

Duke Authors

Cited Authors

  • Settles, DL; Cihak, RA; Erickson, HP

Published Date

  • February 1996

Published In

Volume / Issue

  • 19 / 2

Start / End Page

  • 147 - 154

PubMed ID

  • 8559162

Pubmed Central ID

  • 8559162

International Standard Serial Number (ISSN)

  • 0148-639X

Digital Object Identifier (DOI)

  • 10.1002/(SICI)1097-4598(199602)19:2<147::AID-MUS4>3.0.CO;2-E

Language

  • eng

Conference Location

  • United States