Absence of T and B lymphocytes modulates dystrophic features in dysferlin deficient animal model
Articolo
Data di Pubblicazione:
2012
Citazione:
Absence of T and B lymphocytes modulates dystrophic features in dysferlin deficient animal model / A. Farini, C. Sitzia, C. Navarro, G. D’Antona, M. Belicchi, D. Parolini, G. Del Fraro, P. Razini, R. Bottinelli, M. Meregalli, Y. Torrente.. - In: EXPERIMENTAL CELL RESEARCH. - ISSN 0014-4827. - 318:10(2012 Mar), pp. 1160-1174.
Abstract:
Dysferlin mutations cause muscular dystrophy (dysferlinopathy) characterized by adult onset
muscle weakness, high serum creatine kinase levels, attenuation of muscle regeneration and a
prominent inflammatory infiltrate. In order to verify the role of lymphocytes and immune cells
on this disease, we generated the Scid/A/J transgenic mice and compared these animals with the
age-matched A/J mice. The absence of T and B lymphocytes in this animal model of dysferlinopathy
resulted in an improvement of the muscle regeneration. Scid/A/J mice showed increased specific
force in the myosin heavy chain 2A-expressing fibers of the diaphragm and abdominal
muscles. Moreover, a partial reduction in complement deposition was observed together with a
diminution in pro-inflammatory M1 macrophages. Consistent with this model, T and B lymphocytes
seem to have a role in the muscle damaging immune response. The knowledge of the involvement
of immune system in the development of dysferlinopathies could represent an
important tool for their rescuing. By studying Scid/blAJ mice, we showed that it could be possible
to modulate the pathological symptoms of these diseases by interfering with different components
of the immune system
Tipologia IRIS:
01 - Articolo su periodico
Keywords:
Dysferlin; Lymphocytes; Scid/A/J mice
Elenco autori:
A. Farini, C. Sitzia, C. Navarro, G. D’Antona, M. Belicchi, D. Parolini, G. Del Fraro, P. Razini, R. Bottinelli, M. Meregalli, Y. Torrente.
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