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Cited by 357

The DOI matches its Crossref record; 4 of the 4 references it cites were found in a registry or an index.

The role of Axin2 in calvarial morphogenesis and craniosynostosis

Open access
Yu, Hsiao-Man Ivy, Jerchow, Boris, Sheu, Tzong-Jen, Liu, Bo, Costantini, Frank, Puzas, J. Edward, Birchmeier, Walter, Hsu, Wei·2005·Development

Abstract

Axin1 and its homolog Axin2/conductin/Axil are negative regulators of the canonical Wnt pathway that suppress signal transduction by promoting degradation of β-catenin. Mice with deletion of Axin1 exhibit defects in axis determination and brain patterning during early embryonic development. We show that Axin2 is expressed in the osteogenic fronts and periosteum of developing sutures during skull morphogenesis. Targeted disruption of Axin2 in mice induces malformations of skull structures, a phenotype resembling craniosynostosis in humans. In the mutants, premature fusion of cranial sutures occurs at early postnatal stages. To elucidate the mechanism of craniosynostosis, we studied intramembranous ossification in Axin2-null mice. The calvarial osteoblast development is significantly affected by the Axin2 mutation. The Axin2mutant displays enhanced expansion of osteoprogenitors, accelerated ossification, stimulated expression of osteogenic markers and increases in mineralization. Inactivation of Axin2 promotes osteoblast proliferation and differentiation in vivo and in vitro. Furthermore, as the mammalian skull is formed from cranial skeletogenic mesenchyme, which is derived from mesoderm and neural crest, our data argue for a region-specific effect of Axin2 on neural crest dependent skeletogenesis. The craniofacial anomalies caused by the Axin2 mutation are mediated through activation of β-catenin signaling, suggesting a novel role for the Wnt pathway in skull morphogenesis.

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Metadata

Authors
Yu, H.-M. I., Jerchow, B., Sheu, T.-J., Liu, B., Costantini, F., Puzas, J. E., Birchmeier, W., & Hsu, W.
Venue
Development
Published
2005-04-15
Volume
132
Issue
8
Pages
1995-2005
ISSN
  • 0950-1991 (ISSN-L)
  • 1477-9129
OpenAlex
W2083574228
Cited by
357
References
4
FWCI
10.08
Type
journal article
Language
en
Open access
bronze

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