Ophir D. Klein, M.D., Ph.D. is Associate Professor in the Departments of Orofacial Sciences and Pediatrics, Chair of the Division of Craniofacial Anomalies, and Director of the Program in Craniofacial and Mesenchymal Biology at UCSF. Dr. Klein's research focuses in large part on understanding the processes underlying craniofacial and dental development. His laboratory uses mouse models to study the mechanisms responsible for the normal and abnormal development of teeth, facial skeleton, and other organs, as well as the regeneration of these organs.
Developmental disorders of the dentition: An update
Article first published online: 4 OCT 2013
© 2013 Wiley Periodicals, Inc.
American Journal of Medical Genetics Part C: Seminars in Medical Genetics
Special Issue: Craniofacial Anomalies: Models, Mechanisms, and Management
Volume 163, Issue 4, pages 318–332, November 2013
How to Cite
2013. Developmental disorders of the dentition: An update. Am J Med Genet Part C Semin Med Genet 163C:318–332., , , , , .
- Issue published online: 24 OCT 2013
- Article first published online: 4 OCT 2013
- NIH. Grant Numbers: DP2-OD00719, R01-DE021420
- California Institute of Regenerative Medicine. Grant Number: RN2-00933
- Grant Agency of the Czech Republic. Grant Number: CZ:GA ČR:GAP305/12/1766
- supernumerary teeth;
Dental anomalies are common congenital malformations that can occur either as isolated findings or as part of a syndrome. This review focuses on genetic causes of abnormal tooth development and the implications of these abnormalities for clinical care. As an introduction, we describe general insights into the genetics of tooth development obtained from mouse and zebrafish models. This is followed by a discussion of isolated as well as syndromic tooth agenesis, including Van der Woude syndrome (VWS), ectodermal dysplasias (EDs), oral-facial-digital (OFD) syndrome type I, Rieger syndrome, holoprosencephaly, and tooth anomalies associated with cleft lip and palate. Next, we review delayed formation and eruption of teeth, as well as abnormalities in tooth size, shape, and form. Finally, isolated and syndromic causes of supernumerary teeth are considered, including cleidocranial dysplasia and Gardner syndrome. © 2013 Wiley Periodicals, Inc.