Mesoangioblasts have been characterized as a population of vessel-associated stem cells able to differentiate into several mesodermal cell types, including skeletal muscle. Here, we report that the paired box transcription factor Pax3 plays a crucial role in directing mouse mesoangioblasts toward skeletal myogenesis in vitro and in vivo. Mesoangioblasts isolated from the aorta of Pax3 null embryos are severely impaired in skeletal muscle differentiation, whereas most other differentiation programs are not affected by the absence of Pax3. Moreover, Pax3-/- null mesoangioblasts failed to rescue the myopathic phenotype of the α-sarcoglycan mutant mouse. In contrast, mesoangioblasts from Pax3 gain of function, Pax3PAX3-FKHR/+, mice display enhanced myogenesis in vitro and are more efficient in regenerating new muscle fibers in this model of muscular dystrophy. These data demonstrate that Pax3 is required for the differentiation of mesoangioblast stem cells into skeletal muscle, in keeping with its role in orchestrating entry into the myogenic program.

Messina, G., Sirabella, D., Monteverde, S., Galvez, B., Tonlorenzi, R., Schnapp, E., et al. (2009). Skeletal Muscle Differentiation Of Embryonic Mesoangioblasts Requires Pax3 Activity. STEM CELLS, 27(1), 157-164 [10.1634/stemcells.2008-0503].

Skeletal Muscle Differentiation Of Embryonic Mesoangioblasts Requires Pax3 Activity

BRUNELLI, SILVIA;
2009

Abstract

Mesoangioblasts have been characterized as a population of vessel-associated stem cells able to differentiate into several mesodermal cell types, including skeletal muscle. Here, we report that the paired box transcription factor Pax3 plays a crucial role in directing mouse mesoangioblasts toward skeletal myogenesis in vitro and in vivo. Mesoangioblasts isolated from the aorta of Pax3 null embryos are severely impaired in skeletal muscle differentiation, whereas most other differentiation programs are not affected by the absence of Pax3. Moreover, Pax3-/- null mesoangioblasts failed to rescue the myopathic phenotype of the α-sarcoglycan mutant mouse. In contrast, mesoangioblasts from Pax3 gain of function, Pax3PAX3-FKHR/+, mice display enhanced myogenesis in vitro and are more efficient in regenerating new muscle fibers in this model of muscular dystrophy. These data demonstrate that Pax3 is required for the differentiation of mesoangioblast stem cells into skeletal muscle, in keeping with its role in orchestrating entry into the myogenic program.
Articolo in rivista - Articolo scientifico
Mesoangioblasts; Muscular myopathies; Myogenesis; Pax3;
English
2009
27
1
157
164
none
Messina, G., Sirabella, D., Monteverde, S., Galvez, B., Tonlorenzi, R., Schnapp, E., et al. (2009). Skeletal Muscle Differentiation Of Embryonic Mesoangioblasts Requires Pax3 Activity. STEM CELLS, 27(1), 157-164 [10.1634/stemcells.2008-0503].
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/6133
Citazioni
  • Scopus 30
  • ???jsp.display-item.citation.isi??? 29
Social impact