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doi: 10.1242/10.1242/dev.00581
DEVELOPMENT AND DISEASE |
1 Howard Hughes Medical Institute and Department of Biological Chemistry,
University of California, Los Angeles, CA 90095-1662, USA
2 Victor Goodhill Ear Center, Head and Neck Surgery Division, University of
California, Los Angeles, CA 90095-1794, USA
3 Department of Cell Biology, Duke University Medical Center, Durham, NC 272710,
USA
4 Samuel Lunenfeld Research Institute, Mount Sinai Hospital, University of
Toronto, Toronto, M5G 1X5, Canada
Author for correspondence (e-mail:
bachiller{at}hnsurg.medsch.ucla.edu)
Accepted 24 April 2003
The chordin/Bmp system provides one of the best examples of extracellular signaling regulation in animal development. We present the phenotype produced by the targeted inactivation of the chordin gene in mouse. Chordin homozygous mutant mice show, at low penetrance, early lethality and a ventralized gastrulation phenotype. The mutant embryos that survive die perinatally, displaying an extensive array of malformations that encompass most features of DiGeorge and Velo-Cardio-Facial syndromes in humans. Chordin secreted by the mesendoderm is required for the correct expression of Tbx1 and other transcription factors involved in the development of the pharyngeal region. The chordin mutation provides a mouse model for head and neck congenital malformations that frequently occur in humans and suggests that chordin/Bmp signaling may participate in their pathogenesis.
Key words: Chordin, Bmp, Tbx1, Fgf8, DiGeorge, Pharyngeal endoderm, Ventralization, Neural crest, Patterning, Persistent truncus arteriosus, Mouse
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