Title: MyoD expression restores defective myogenic differentiation of human mesoangioblasts from inclusion-body myositis muscle
Authors: Morosetti, Roberta ×
Mirabella, Massimiliano
Gliubizzi, Carla
Broccolini, Aldobrando
De Angelis, Luciana
Tagliafico, Enrico
Sampaolesi, Maurilio
Gidaro, Teresa
Papacci, Manuela
Roncaglia, Enrica
Rutella, Sergio
Ferrari, Stefano
Tonali, Pietro Attilio
Ricci, Enzo
Cossu, Giulio #
Issue Date: Nov-2006
Series Title: Proceedings of the National Academy of Sciences of the United States of America vol:103 issue:45 pages: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.
ISSN: 0027-8424
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Interdepartemental Stem Cell Institute (-)
× corresponding author
# (joint) last author

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