Tags

Type your tag names separated by a space and hit enter

Molecular mechanisms in calvarial bone and suture development, and their relation to craniosynostosis.
Eur J Orthod 2003; 25(2):139-48EJ

Abstract

The development and growth of the skull is a co-ordinated process involving many different tissues that interact with each other to form a complex end result. When normal development is disrupted, debilitating pathological conditions, such as craniosynostosis (premature calvarial suture fusion) and cleidocranial dysplasia (delayed suture closure), can result. It is known that mutations in the fibroblast growth factor receptors 1, 2, and 3(FGFR1, 2, and 3), as well as the transcription factors MSX2 and TWIST cause craniosynostosis, and that mutations in the transcription factor RUNX2 (CBFA1) cause cleidocranial dysplasia. However, relatively little is known about the development of the calvaria: where and when these genes are active during normal calvarial development, how these genes may interact in the developing calvaria, and the disturbances that may occur to cause these disorders. In this work an attempt has been made to address some of these questions from a basic biological perspective. The expression patterns of the above-mentioned genes in the developing mouse skull are detailed. The microdissection and in vitro culture techniques have begun the task of identifying Fgfrs, Msx2, and Twist interacting in intricate signalling pathways that if disrupted could lead to craniosynostosis.

Authors+Show Affiliations

Developmental Biology Programme, Institute of Biotechnology, and Department of Pedodontics and Orthodontics, Institute of Dentistry, University of Helsinki, Finland.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

12737212

Citation

Rice, David P C., et al. "Molecular Mechanisms in Calvarial Bone and Suture Development, and Their Relation to Craniosynostosis." European Journal of Orthodontics, vol. 25, no. 2, 2003, pp. 139-48.
Rice DP, Rice R, Thesleff I. Molecular mechanisms in calvarial bone and suture development, and their relation to craniosynostosis. Eur J Orthod. 2003;25(2):139-48.
Rice, D. P., Rice, R., & Thesleff, I. (2003). Molecular mechanisms in calvarial bone and suture development, and their relation to craniosynostosis. European Journal of Orthodontics, 25(2), pp. 139-48.
Rice DP, Rice R, Thesleff I. Molecular Mechanisms in Calvarial Bone and Suture Development, and Their Relation to Craniosynostosis. Eur J Orthod. 2003;25(2):139-48. PubMed PMID: 12737212.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Molecular mechanisms in calvarial bone and suture development, and their relation to craniosynostosis. AU - Rice,David P C, AU - Rice,Ritva, AU - Thesleff,Irma, PY - 2003/5/10/pubmed PY - 2003/7/24/medline PY - 2003/5/10/entrez SP - 139 EP - 48 JF - European journal of orthodontics JO - Eur J Orthod VL - 25 IS - 2 N2 - The development and growth of the skull is a co-ordinated process involving many different tissues that interact with each other to form a complex end result. When normal development is disrupted, debilitating pathological conditions, such as craniosynostosis (premature calvarial suture fusion) and cleidocranial dysplasia (delayed suture closure), can result. It is known that mutations in the fibroblast growth factor receptors 1, 2, and 3(FGFR1, 2, and 3), as well as the transcription factors MSX2 and TWIST cause craniosynostosis, and that mutations in the transcription factor RUNX2 (CBFA1) cause cleidocranial dysplasia. However, relatively little is known about the development of the calvaria: where and when these genes are active during normal calvarial development, how these genes may interact in the developing calvaria, and the disturbances that may occur to cause these disorders. In this work an attempt has been made to address some of these questions from a basic biological perspective. The expression patterns of the above-mentioned genes in the developing mouse skull are detailed. The microdissection and in vitro culture techniques have begun the task of identifying Fgfrs, Msx2, and Twist interacting in intricate signalling pathways that if disrupted could lead to craniosynostosis. SN - 0141-5387 UR - https://www.unboundmedicine.com/medline/citation/12737212/Molecular_mechanisms_in_calvarial_bone_and_suture_development_and_their_relation_to_craniosynostosis_ L2 - https://academic.oup.com/ejo/article-lookup/doi/10.1093/ejo/25.2.139 DB - PRIME DP - Unbound Medicine ER -