Enamel formation and amelogenesis imperfecta

Jan C.C. Hu, Yong Hee P. Chun, Turki Al Hazzazzi, James P. Simmer

Research output: Contribution to journalReview article

142 Scopus citations

Abstract

Dental enamel is the epithelial-derived hard tissue covering the crowns of teeth. It is the most highly mineralized and hardest tissue in the body. Dental enamel is acellular and has no physiological means of repair outside of the protective and remineralization potential provided by saliva. Enamel is comprised of highly organized hydroxyapatite crystals that form in a defined extracellular space, the contents of which are supplied and regulated by ameloblasts. The entire process is under genetic instruction. The genetic control of amelogenesis is poorly understood, but requires the activities of multiple components that are uniquely important for dental enamel formation. Amelogenesis imperfecta (AI) is a collective designation for the variety of inherited conditions displaying isolated enamel malformations, but the designation is also used to indicate the presence of an enamel phenotype in syndromes. Recently, genetic studies have demonstrated the importance of genes encoding enamel matrix proteins in the etiology of isolated AI. Here we review the essential elements of dental enamel formation and the results of genetic analyses that have identified disease-causing mutations in genes encoding enamel matrix proteins. In addition, we provide a fresh perspective on the roles matrix proteins play in catalyzing the biomineralization of dental enamel.

Original languageEnglish (US)
Pages (from-to)78-85
Number of pages8
JournalCells Tissues Organs
Volume186
Issue number1
DOIs
StatePublished - Jul 2007
Externally publishedYes

Keywords

  • Amelogenesis imperfecta
  • Amelogenin
  • Enamel
  • Enamelin
  • Enamelysin
  • Kallikrein 4

ASJC Scopus subject areas

  • Anatomy
  • Histology

Fingerprint Dive into the research topics of 'Enamel formation and amelogenesis imperfecta'. Together they form a unique fingerprint.

Cite this