Sphenoid bone hypoplasia is a skeletal phenotype of cleidocranial dysplasia in a mouse model and patients.
Cleidocranial dysplasia
Osterix
Runx2
Secondary cartilage
Sphenoid bone
Journal
Bone
ISSN: 1873-2763
Titre abrégé: Bone
Pays: United States
ID NLM: 8504048
Informations de publication
Date de publication:
03 2019
03 2019
Historique:
received:
10
08
2018
revised:
31
10
2018
accepted:
31
10
2018
pubmed:
6
11
2018
medline:
20
2
2020
entrez:
5
11
2018
Statut:
ppublish
Résumé
Cleidocranial dysplasia (CCD) is an autosomal dominant disorder caused by heterozygous mutations in RUNX2. Affected individuals exhibit delayed maturation or hypoplasia in various bones, mainly including those formed by intramembranous ossification. Although several reports described deformation of the sphenoid bone in CCD patients, details of the associated changes have not been well documented. Most parts of the sphenoid bone are formed by endochondral ossification; however, the medial pterygoid process is formed by intramembranous ossification associated with secondary cartilage. We first investigated histological changes in the medial pterygoid process during different developmental stages in Runx2
Identifiants
pubmed: 30391578
pii: S8756-3282(18)30421-6
doi: 10.1016/j.bone.2018.10.028
pii:
doi:
Substances chimiques
Core Binding Factor Alpha 1 Subunit
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
176-186Informations de copyright
Copyright © 2018 Elsevier Inc. All rights reserved.