title: The Development of the Calvarial Bones and Sutures and the Pathophysiology of Craniosynostosis.
abstract: The skull vault is a complex, exquisitely patterned structure that plays a variety of key roles in vertebrate life, ranging from the acquisition of food to the support of the sense organs for hearing, smell, sight, and taste. During its development, it must meet the dual challenges of protecting the brain and accommodating its growth. The bones and sutures of the skull vault are derived from cranial neural crest and head mesoderm. The frontal and parietal bones develop from osteogenic rudiments in the supraorbital ridge. The coronal suture develops from a group of Shh-responsive cells in the head mesoderm that are collocated, with the osteogenic precursors, in the supraorbital ridge. The osteogenic rudiments and the prospective coronal suture expand apically by cell migration. A number of congenital disorders affect the skull vault. Prominent among these is craniosynostosis, the fusion of the bones at the sutures. Analysis of the pathophysiology underling craniosynostosis has identified a variety of cellular mechanisms, mediated by a range of signaling pathways and effector transcription factors. These cellular mechanisms include loss of boundary integrity, altered sutural cell specification in embryos, and loss of a suture stem cell population in adults. Future work making use of genome-wide transcriptomic approaches will address the deep structure of regulatory interactions and cellular processes that unify these seemingly diverse mechanisms.
authors.affiliation: Department of Biochemistry and Molecular Biology, USC Norris Comprehensive Cancer Center, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
authors.forename: Mamoru
authors.initials: M
authors.lastname: Ishii
authors.affiliation: Department of Biochemistry and Molecular Biology, USC Norris Comprehensive Cancer Center, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
authors.forename: Jingjing
authors.initials: J
authors.lastname: Sun
authors.affiliation: Department of Biochemistry and Molecular Biology, USC Norris Comprehensive Cancer Center, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
authors.forename: Man-Chun
authors.initials: MC
authors.lastname: Ting
authors.affiliation: Department of Biochemistry and Molecular Biology, USC Norris Comprehensive Cancer Center, Keck School of Medicine, University of Southern California, Los Angeles, California, USA. Electronic address: maxson@usc.edu.
authors.forename: Robert E
authors.initials: RE
authors.lastname: Maxson
mesh_terms: D000818:Animals; D003393:Cranial Sutures / Q000196:embryology* / Q000378:metabolism; D003398:Craniosynostoses / Q000196:embryology* / Q000235:genetics / Q000503:physiopathology; D018507:Gene Expression Regulation, Developmental; D006801:Humans; D008648:Mesoderm / Q000196:embryology / Q000378:metabolism; D009024:Morphogenesis; D009432:Neural Crest / Q000196:embryology / Q000378:metabolism; D015398:Signal Transduction / Q000235:genetics; D012886:Skull / Q000196:embryology* / Q000378:metabolism
keywords: Calvaria; Cranial sutures; Craniosynostosis; Frontal bone; Head mesoderm; Neural crest; Osteoblast; Parietal bone