Diseases of the tooth: the genetic and molecular basis of inherited anomalies affecting the dentition.

[dentinogenesis imperfecta]

In humans , inherited variation in the number , size , and shape of teeth within the dentitions are relatively common , while rarer defects of hard tissue formation , including amelogenesis and dentinogenesis imperfecta , and problems associated with tooth eruption are also seen . In many cases , these anomalies occur in isolation , but they can also present as a feature of numerous well-characterized developmental syndromes . Complex reiterative signaling between the epithelium and mesenchyme is a feature of normal tooth development in the embryo , occurring from early patterning through morphogenesis , hard tissue formation and during root development . Significant events also occur during postnatal development of the dentition , including hard tissue maturation and tooth eruption . In the last decade , advances in human and mouse genetics have meant that in many cases candidate genes have been identified for these anomalies . These genes have provided a useful platform for developmental biologists , allowing them to begin elucidating how these signals interact to generate a functional dentition and understand the mechanisms underlying many of the anomalies that are seen in human populations . In this article , we review current concepts relating to the developmental biology of tooth number , size , and shape , formation of the dental hard tissues and eruption of the tooth into the oral cavity . We will focus on the molecular mechanisms underlying these processes in both health and disease .

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Diseases presenting "allowing them to begin elucidating how these signals interact to generate a functional dentition and understand the mechanisms underlying many of the anomalies that are seen in human populations" symptom

dentinogenesis imperfecta

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