Journal
DEVELOPMENTAL DYNAMICS
Volume 235, Issue 5, Pages 1441-1447Publisher
WILEY
DOI: 10.1002/dvdy.20598
Keywords
lip; palate; Interferon regulatory factor 6; Van der Woude syndrome; cleft lip; cleft palate
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Funding
- NIDCR NIH HHS [DE015207, R01 DE013681, P50 DE016215, P50-DE016215] Funding Source: Medline
- Wellcome Trust [064732] Funding Source: Medline
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Development of the lip and palate involves a complex series of events that are frequently disturbed resulting in the congenital anomalies cleft lip and cleft palate. Van der Woude syndrome (VWS) is an autosomal dominant disorder that is characterised by cleft lip, cleft palate, lower lip pits, and hypodontia. VWS arises as the result of mutations in the gene encoding interferon regulatory factor 6 (IRF6). To provide insights into the role of IRF6 during embryogenesis, we have analysed the expression of this molecule during mouse and chick facial development. Irf6 was expressed in the ectoderm covering the facial processes during their fusion to form the upper lip and primary palate in both mouse and chick. However, while Irf6 was expressed in the medial edge epithelia of the developing secondary palate of the mouse, which fuses as in man, Irf6 was not expressed in the medial edge epithelia of the naturally cleft chick secondary palate. Similarly, Irf6 was found to be down-regulated in the medial edge epithelia of transforming growth factor beta3-null mice, which also exhibit cleft palate. Together, these results support a role for IRF6 during the fusion events that occur during development of the lip and palate. Developmental Dynamics 235:1441-1447, 2006. (c) 2005 Wiley-Liss, Inc.
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