Temporal and spatial mRNA expression patterns of TGF-β1, 2, 3 and TβRI, II, III in skeletal muscles of mdx mice

Lan Zhou, John D. Porter, Georgiana Cheng, Bendi Gong, Denise A. Hatala, Anita P. Merriam, Xiaohua Zhou, Jill A. Rafael, Henry J. Kaminski

Research output: Contribution to journalArticlepeer-review

64 Scopus citations

Abstract

To address potential regulatory roles of TGF-β1 in muscle inflammation and fibrosis associated with dystrophin deficiency, we performed quantitative RT-PCR and in situ hybridization to characterize the temporal and spatial mRNA expression patterns of TGF-β1 and other TGF-β subfamily members, TGF-β2 and TGF-β3, as well as their receptors, in quadriceps and diaphragm muscles of mdx mice. TGF-β1 mRNA was markedly upregulated in the endomysial inflammatory cells and regenerating fibers of mdx quadriceps and diaphragm, with the mRNA levels correlated with the degree of endomysial inflammation. Upregulation of TGF-β2, β3, and their receptors was also appreciated but to a much lesser degree. While high levels of TGF-β1 mRNA remained in the aging mdx quadriceps but not the diaphragm, progressive fibrosis only occurred in the diaphragm. Our data support a regulatory role for TGF-β1 in muscle inflammation in mdx mice. It also suggests different susceptibility of quadriceps and diaphragm muscles to fibrosis induced by TGF-β1 signaling pathway.

Original languageEnglish (US)
Pages (from-to)32-38
Number of pages7
JournalNeuromuscular Disorders
Volume16
Issue number1
DOIs
StatePublished - Jan 2006

Keywords

  • Duchenne muscular dystrophy
  • Dystrophin
  • TGF-β
  • TGF-β receptors
  • mdx Mice

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Clinical Neurology
  • Genetics(clinical)

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