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Proc Natl Acad Sci U S A. 2007 Mar 13;104(11):4443-8. Epub 2007 Mar 7.

RBP-J (Rbpsuh) is essential to maintain muscle progenitor cells and to generate satellite cells.

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  • 1Max Delbrück Center for Molecular Medicine, Robert Rössle Strasse 10, 13125 Berlin, Germany.

Abstract

In the developing muscle, a pool of myogenic progenitor cells is formed and maintained. These resident progenitors provide a source of cells for muscle growth in development and generate satellite cells in the perinatal period. By the use of conditional mutagenesis in mice, we demonstrate here that the major mediator of Notch signaling, the transcription factor RBP-J, is essential to maintain this pool of progenitor cells in an undifferentiated state. In the absence of RBP-J, these cells undergo uncontrolled myogenic differentiation, leading to a depletion of the progenitor pool. This results in a lack of muscle growth in development and severe muscle hypotrophy. In addition, satellite cells are not formed late in fetal development in conditional RBP-J mutant mice. We conclude that RBP-J is required in the developing muscle to set aside proliferating progenitors and satellite cells.

PMID:
17360543
[PubMed - indexed for MEDLINE]
PMCID:
PMC1815471
Free PMC Article
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