MyoD expression restores defective myogenic differentiation of human mesoangioblasts from inclusion-body myositis muscle.

Massimiliano Mirabella, Aldobrando Broccolini, Enzo Ricci, Roberta Morosetti, Carla Gliubizzi, Teresa Gidaro, Manuela Papacci, Sergio Rutella, Pietro Attilio Tonali

Risultato della ricerca: Contributo in rivistaArticolo in rivistapeer review

59 Citazioni (Scopus)

Abstract

Inflammatory myopathies (IM) are acquired diseases of skeletal muscle comprising dermatomyositis (DM), polymyositis (PM), and inclusion-body myositis (IBM). Immunosuppressive therapies, usually beneficial for DM and PM, are poorly effective in IBM. We report the isolation and characterization of mesoangioblasts, vessel-associated stem cells, from diagnostic muscle biopsies of IM. The number of cells isolated, proliferation rate and lifespan, markers expression, and ability to differentiate into smooth muscle do not differ among normal and IM mesoangioblasts. At variance with normal, DM and PM mesoangioblasts, cells isolated from IBM, fail to differentiate into skeletal myotubes. These data correlate with lack in connective tissue of IBM muscle of alkaline phosphatase (ALP)-positive cells, conversely dramatically increased in PM and DM. A myogenic inhibitory basic helix-loop-helix factor B3 is highly expressed in IBM mesoangioblasts. Indeed, silencing this gene or overexpressing MyoD rescues the myogenic defect of IBM mesoangioblasts, opening novel cell-based therapeutic strategies for this crippling disorder.
Lingua originaleEnglish
pagine (da-a)16995-17000
Numero di pagine6
RivistaProceedings of the National Academy of Sciences of the United States of America
Volume2006
Stato di pubblicazionePubblicato - 2006

Keywords

  • IBM
  • mesoangioblasts
  • muscle diseases
  • stem cells

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