title: Genetic Disorders of Dental Development: Tales from the Bony Crypt.
abstract: The ebb and flow of genetic influence relative to the understanding of craniofacial and dental disorders has evolved into a tacit acceptance of the current genetic paradigm. This review explores the science behind craniofacial and dental disorders through the lens of recent past and current findings and using tooth agenesis as a model of advances in craniofacial genetics.
  
  
  
  Contemporary studies of craniofacial biology takes advantage of the technological resources stemming from the genomic and post-genomic eras. Emerging data highlights the role of key genes and the epigenetic landscape controlling these genes, in causing dentofacial abnormalities. We also report here a novel Glu78FS MSX1 mutation in one family segregating an autosomal dominant form of severe tooth agenesis as an illustration of an evolving theme, i.e., different mutations in the same gene can result in a spectrum of dentofacial phenotypic severity. The future of clinical therapeutics will benefit from advances in genetics and molecular biology that refine the genotype-phenotype correlation. Indeed, the past century suggests a continued convergence of genetic science in the practice of clinical dentistry.
authors.affiliation: Department of Orthodontics, School of Dentistry, University of North Carolina at Chapel Hill, CB #7450, Chapel Hill, NC, 27599-7450, USA. sylvia_frazier-bowers@unc.edu.
authors.forename: Sylvia A
authors.initials: SA
authors.lastname: Frazier-Bowers
authors.affiliation: Department of Oral Health Sciences, Faculty of Dentistry, University of British Columbia, JBM-184 - 2199 Wesbrook Mall, Vancouver, BC, V6T 1Z3, Canada.
authors.forename: Siddharth R
authors.initials: SR
authors.lastname: Vora
mesh_terms: D000848:Anodontia / Q000235:genetics*; D044127:Epigenesis, Genetic; D016368:Frameshift Mutation; D018507:Gene Expression Regulation, Developmental / Q000235:genetics*; D006801:Humans; D050835:Interferon Regulatory Factors / Q000235:genetics; D051957:MSX1 Transcription Factor / Q000235:genetics; D009154:Mutation; D010641:Phenotype; D014096:Tooth, Supernumerary / Q000235:genetics*
chemical_list: C109803:IRF6 protein, human; D050835:Interferon Regulatory Factors; D051957:MSX1 Transcription Factor; C484083:MSX1 protein, human
keywords: Craniofacial disorders; Dental disorders; Epigenetics; Genetics; Tooth agenesis