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RESEARCH COMMUNICATION
signaling in neural crest stem cells leads to multiple defects reminiscent of DiGeorge syndrome
1 Institute of Cell Biology, Department of Biology, Swiss Federal Institute of Technology, ETH-Hönggerberg, Zurich, CH-8093, Switzerland; 2 Research Laboratory for Calcium Metabolism, University Hospital Balgrist, University of Zurich, Zurich, CH-8008, Switzerland; 3 Institute of Experimental Immunology, University Hospital, University of Zurich, Zurich, CH-8091, Switzerland; 4 Departments for Molecular Medicine and Gene Therapy, Lund University, Malmö, S-22184, Sweden
Specific inactivation of TGF
signaling in neural crest stem cells (NCSCs) results in cardiovascular defects and thymic, parathyroid, and craniofacial anomalies. All these malformations characterize DiGeorge syndrome, the most common microdeletion syndrome in humans. Consistent with a role of TGF in promoting non-neural lineages in NCSCs, mutant neural crest cells migrate into the pharyngeal apparatus but are unable to acquire non-neural cell fates. Moreover, in neural crest cells, TGF signaling is both sufficient and required for phosphorylation of CrkL, a signal adaptor protein implicated in the development of DiGeorge syndrome. Thus, TGF signal modulation in neural crest differentiation might play a crucial role in the etiology of DiGeorge syndrome.
[Keywords: Neural crest; TGF; CrkL; Src kinase; fate decision; pharyngeal apparatus; DiGeorge syndrome]
Received July 16, 2004; revised version accepted December 23, 2004.
Article and publication are at http://www.genesdev.org/cgi/doi/10.1101/gad.317405.
5 These authors contributed equally to this work.
6 Corresponding author.
E-MAIL lukas.sommer{at}cell.biol.ethz.ch; FAX 41-1-633-10-69.
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