title: BCL11B regulates sutural patency in the mouse craniofacial skeleton.
abstract: The transcription factor BCL11B plays essential roles during development of the immune, nervous, and cutaneous systems. Here we show that BCL11B is expressed in both osteogenic and sutural mesenchyme of the developing craniofacial complex. Bcl11b(-/-) mice exhibit increased proliferation of osteoprogenitors, premature osteoblast differentiation, and enhanced skull mineralization leading to synostoses of facial and calvarial sutures. Ectopic expression of Fgfr2c, a gene implicated in craniosynostosis in mice and humans, and that of Runx2 was detected within the affected sutures of Bcl11b(-/-) mice. These data suggest that ectopic expression of Fgfr2c in the sutural mesenchyme, without concomitant changes in the expression of FGF ligands, appears to induce the RUNX2-dependent osteogenic program and craniosynostosis in Bcl11b(-/-) mice.
authors.affiliation: Department of Pharmaceutical Sciences, College of Pharmacy, USA.
authors.forename: Kateryna
authors.initials: K
authors.lastname: Kyrylkova
authors.affiliation: Skeletal Biology Laboratory, School of Biological and Population Health Sciences, Oregon State University, Corvallis, OR 97331, USA.
authors.forename: Urszula T
authors.initials: UT
authors.lastname: Iwaniec
authors.affiliation: Department of Pharmaceutical Sciences, College of Pharmacy, USA.
authors.forename: Kenneth A
authors.initials: KA
authors.lastname: Philbrick
authors.affiliation: Department of Pharmaceutical Sciences, College of Pharmacy, USA; Department of Integrative Biosciences, Oregon Health & Science University, Portland, OR 97201, USA. Electronic address: mark.leid@oregonstate.edu.
authors.forename: Mark
authors.initials: M
authors.lastname: Leid
mesh_terms: D000818:Animals; D050659:Core Binding Factor Alpha 1 Subunit / Q000502:physiology; D003393:Cranial Sutures / Q000196:embryology*; D003398:Craniosynostoses / Q000000981:diagnostic imaging / Q000235:genetics / Q000503:physiopathology; D005147:Facial Bones / Q000000981:diagnostic imaging / Q000196:embryology* / Q000473:pathology; D018507:Gene Expression Regulation, Developmental; D008648:Mesoderm / Q000378:metabolism; D051379:Mice; D018345:Mice, Knockout; D009432:Neural Crest / Q000166:cytology; D010006:Osteoblasts / Q000378:metabolism / Q000473:pathology; D051497:Receptor, Fibroblast Growth Factor, Type 2 / Q000502:physiology; D012097:Repressor Proteins / Q000172:deficiency / Q000235:genetics / Q000502:physiology*; D012886:Skull / Q000000981:diagnostic imaging / Q000196:embryology* / Q000473:pathology; D025521:Tumor Suppressor Proteins / Q000172:deficiency / Q000235:genetics / Q000502:physiology*
chemical_list: C511143:Bcl11b protein, mouse; D050659:Core Binding Factor Alpha 1 Subunit; D012097:Repressor Proteins; C493716:Runx2 protein, mouse; D025521:Tumor Suppressor Proteins; C496354:Fgfr2 protein, mouse; D051497:Receptor, Fibroblast Growth Factor, Type 2
keywords: BCL11B/CTIP2; Craniofacial development; Craniosynostosis; Fgfr2c; Midfacial hypoplasia; Runx2; Twist1