title: Osterix is required for cranial neural crest-derived craniofacial bone formation.
abstract: Osx plays essential roles in regulating osteoblast and chondrocyte differentiation, and bone formation during mouse skeletal development. However, many questions remain regarding the requirement for Osx in different cell lineages. In this study, we asked whether Osx is required for craniofacial bone formation derived from cranial neural crest (CNC) cells. The Osx gene was conditionally inactivated in CNC-derived cells using a Wnt1-Cre recombination system. Neural crest-specific inactivation of Osx resulted in the complete absence of intramembranous skeletal elements derived from the CNC, and CNC-derived endochondral skeletal elements were also affected by Osx inactivation. Interestingly, Osx inactivated CNC-derived cells, which were recapitulated by lacZ expression, occupied the same regions of craniofacial skeletal elements as observed for controls. However, cells lost their osteogenic ability to differentiate into functional osteoblasts by Osx inactivation. These results suggest that Osx is important for craniofacial bone formation by CNC-derived cells. This finding provides novel insights of the regulation of craniofacial development by the gene network and transcription factors, and the understanding of human diseases caused by neural crest developmental abnormalities.
authors: Department of Molecular Medicine, Cell and Matrix Research Institute, BK21 Medical Education Program for Human Resources, Kyungpook National University School of Medicine, Daegu, Republic of Korea.
authors: Wook-Young
authors: WY
authors: Baek
authors: Young-Ji
authors: YJ
authors: Kim
authors: Benoit
authors: B
authors: de Crombrugghe
authors: Jung-Eun
authors: JE
authors: Kim
mesh_terms: D000818:Animals; D019465:Craniofacial Abnormalities / Q000235:genetics* / Q000473:pathology; D005147:Facial Bones / Q000002:abnormalities / Q000196:embryology* / Q000473:pathology; D020868:Gene Silencing; D019426:Integrases / Q000235:genetics; D051379:Mice; D008822:Mice, Transgenic; D009432:Neural Crest / Q000002:abnormalities*; D010012:Osteogenesis / Q000235:genetics*; D000076182:Sp7 Transcription Factor; D014157:Transcription Factors / Q000235:genetics / Q000502:physiology*; D051155:Wnt1 Protein / Q000235:genetics
chemical_list: D000076182:Sp7 Transcription Factor; C479466:Sp7 protein, mouse; D014157:Transcription Factors; D051155:Wnt1 Protein; C495246:Wnt1 protein, mouse; C045073:Cre recombinase; D019426:Integrases