Cranial suture biology and dental development: genetic and clinical perspectives.

[dentin dysplasia]

Premature fusion of the calvarial bones at the sutures , or craniosynostosis (CS ), is a relatively common birth defect (1 :2000 -3000 ) frequently associated with limb deformity . Patients with CS may present oral defects , such as cleft soft palate , hypodontia , hyperdontia , and delayed tooth eruption , but also unusual associations of major dental anomalies such as taurodontism , microdontia , multiple dens invaginatus , and dentin dysplasia . The list of genes that are involved in CS includes those coding for the different fibroblast growth factor receptors and a ligand of ephrin receptors , but also genes encoding transcription factors , such as MSX 2 and TWIST . Most of these genes are equally involved in odontogenesis , providing a pausible explanation for clinical associations of CS with dental agenesis or tooth malformations . On the basis of the present knowledge on genes and transcription factors that are involved in craniofacial morphogenesis , and from dental clinics of CS syndromes , the molecular mechanisms that control suture formation and suture closure are expected to play key roles in patterning events and development of teeth . The purpose of this article is to review and merge the recent advances in the field of suture research at the genetic and cellular levels with those of tooth development , and to apply them to the dental clinics of CS syndromes . These new perspectives and future challenges in the field of both dental clinics and molecular genetics , more in particular the identification of possible candidate genes involved in both CS and dental defects , are discussed .

Diseases
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Diseases presenting "premature fusion of the calvarial bones at the sutures" symptom

dentin dysplasia

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