This is a proposal to establish a Craniofacial Anomalies Research Center at the University of Pennsylvania. The Center will Bring together investigators from the School of Medicine, School of Dental Medicine, Department of Biology, Children's Hospital of Philadelphia, and Fox Chase Cancer Center to work on four projects which will have a central theme of applying molecular biology to normal and abnormal cranioacial development. In the first project, ibological and molecular aspects of a cleft palate susceptibility gene, Cps-1, on chromosome 17 of the mouse will be investigated. The second project will investigate mechanisms of regulation of the gene for amelogenin in mice in order to gain a better iunderstanding of how enamel organ development and enamel matrix synthesis is controlled. In the third project, the X-linked gene for Nance-Horan syndrome will be investigated. Patients affected by this disorder frequently have both cataracts and abnormal teeth, and these experiments will seek to determine whether both of these conditions are associated with the same genetic locus. The fourth project will investigate the molecular genetic bases for Treacher Collins ssyndrome and nonsyndromic orofacial clefts. The Center will include an administrative core and a molecular biology core. Progress will be an administrative core and a molecular biology core. Progress will continuously monitored by both an internal and external advisory committee.
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