Abnormal connective tissue proliferation following muscle degeneration is a major pathological feature of Duchenne muscular dystrophy (DMD), a genetic myopathy due to lack of the sarcolemmal dystrophin protein. Since this fibrotic proliferation is likely to be a major obstacle to the efficacy of future therapies, research is needed to understand and prevent the fibrotic process in order to develop an effective treatment. Murine muscular dystrophy (mdx) is genetically homologous to DMD, and histopatological alterations are comparable to those of the muscles of patients with DMD. To investigate the development of fibrosis, we bred the mdx mouse with the scid immunodepressed mouse and analysed fibrosis histologically; we used ELISA analysis to determine TGF-β1 expression. Significant reduction of fibrosis and TGF-β1 expression was found in the muscles of the scid/mdx mice. However, we observed similar centrally located nuclei, necrosis, muscle degeneration and muscle force compared to the mdx animals. These data demonstrate a correlation between the absence of B and T lymphocytes and loss of fibrosis accompanied by reduction of TGF-β1, suggesting the importance of modulation of the immune system in DMD. Copyright

T and b lymphocyte depletion has a marked effect on the fibrosis of dystrophic skeletal muscles in the scid/mdx mouse / A. Farini, M. Meregalli, M. Belicchi, M. Battistelli, D. Parolini, G. D'Antona, M. Gavina, L. Ottoboni, G. Constantin, R. Bottinelli, Y. Torrente. - In: JOURNAL OF PATHOLOGY. - ISSN 0022-3417. - 213:2(2007 Jul 31), pp. 229-238.

T and b lymphocyte depletion has a marked effect on the fibrosis of dystrophic skeletal muscles in the scid/mdx mouse

A. Farini;M. Meregalli;M. Belicchi;D. Parolini;G. D'Antona;M. Gavina;Y. Torrente
2007-07-31

Abstract

Abnormal connective tissue proliferation following muscle degeneration is a major pathological feature of Duchenne muscular dystrophy (DMD), a genetic myopathy due to lack of the sarcolemmal dystrophin protein. Since this fibrotic proliferation is likely to be a major obstacle to the efficacy of future therapies, research is needed to understand and prevent the fibrotic process in order to develop an effective treatment. Murine muscular dystrophy (mdx) is genetically homologous to DMD, and histopatological alterations are comparable to those of the muscles of patients with DMD. To investigate the development of fibrosis, we bred the mdx mouse with the scid immunodepressed mouse and analysed fibrosis histologically; we used ELISA analysis to determine TGF-β1 expression. Significant reduction of fibrosis and TGF-β1 expression was found in the muscles of the scid/mdx mice. However, we observed similar centrally located nuclei, necrosis, muscle degeneration and muscle force compared to the mdx animals. These data demonstrate a correlation between the absence of B and T lymphocytes and loss of fibrosis accompanied by reduction of TGF-β1, suggesting the importance of modulation of the immune system in DMD. Copyright
Duchenne muscular dystrophy (DMD); Fibrosis; MDX; SCID
Settore MED/26 - Neurologia
JOURNAL OF PATHOLOGY
Article (author)
File in questo prodotto:
Non ci sono file associati a questo prodotto.
Pubblicazioni consigliate

Caricamento pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/44656
Citazioni
  • ???jsp.display-item.citation.pmc??? 49
  • Scopus 74
  • ???jsp.display-item.citation.isi??? 75
social impact