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

Morosetti, R., Mirabella, M., Gliubizzi, C., Broccolini, A., De Angelis, L., Tagliafico, E., Sampaolesi, M., Gidaro, T., Papacci, M., Roncaglia, E., Rutella, S. ORCID: 0000-0003-1970-7375, Ferrari, S., Tonali, P.A., Ricci, E. and Cossu, G., 2006. MyoD expression restores defective myogenic differentiation of human mesoangioblasts from inclusion-body myositis muscle. PNAS: Proceedings of the National Academy of Sciences, 103 (45), pp. 16995-17000. ISSN 0027-8424

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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.

Item Type: Journal article
Publication Title: PNAS: Proceedings of the National Academy of Sciences
Creators: Morosetti, R., Mirabella, M., Gliubizzi, C., Broccolini, A., De Angelis, L., Tagliafico, E., Sampaolesi, M., Gidaro, T., Papacci, M., Roncaglia, E., Rutella, S., Ferrari, S., Tonali, P.A., Ricci, E. and Cossu, G.
Publisher: National Academy of Sciences
Date: November 2006
Volume: 103
Number: 45
ISSN: 0027-8424
Identifiers:
NumberType
10.1073/pnas.0603386103DOI
17077152PubMed ID
Divisions: Schools > School of Science and Technology
Depositing User: Jill Tomkinson
Date Added: 14 Sep 2016 10:46
Last Modified: 09 Jun 2017 14:05
URI: http://irep.ntu.ac.uk/id/eprint/28528

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