MyoD expression restores defective myogenic differentiation of human mesoangioblasts from inclusion-body myositis muscle
Articolo
Data di Pubblicazione:
2006
Citazione:
MyoD expression restores defective myogenic differentiation of human mesoangioblasts from inclusion-body myositis muscle / Roberta Morosetti, Massimiliano Mirabella, Carla Gliubizzi, Aldobrando Broccolini, Luciana De Angelis, Enrico Tagliafico, Maurilio Sampaolesi, Teresa Gidaro, Manuela Papacci, Enrica Roncaglia, Sergio Rutella, Stefano Ferrari, Pietro Attilio Tonali, Enzo Ricci, Giulio Cossu. - In: PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA. - ISSN 0027-8424. - 103:45(2006 Nov 07), pp. 16995-17000.
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.
Tipologia IRIS:
01 - Articolo su periodico
Elenco autori:
Roberta Morosetti, Massimiliano Mirabella, Carla Gliubizzi, Aldobrando Broccolini, Luciana De Angelis, Enrico Tagliafico, Maurilio Sampaolesi, Teresa Gidaro, Manuela Papacci, Enrica Roncaglia, Sergio Rutella, Stefano Ferrari, Pietro Attilio Tonali, Enzo Ricci, Giulio Cossu
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