Twigg Stephen R F, Wilkie Andrew O M
Clinical Genetics Group, Weatherall Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Headington, Oxford OX3 9DS, UK
Clinical Genetics Group, Weatherall Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Headington, Oxford OX3 9DS, UK.
Hum Mol Genet. 2015 Oct 15;24(R1):R50-9. doi: 10.1093/hmg/ddv228. Epub 2015 Jun 17.
Development of the human skull and face is a highly orchestrated and complex three-dimensional morphogenetic process, involving hundreds of genes controlling the coordinated patterning, proliferation and differentiation of tissues having multiple embryological origins. Craniofacial malformations that occur because of abnormal development (including cleft lip and/or palate, craniosynostosis and facial dysostoses), comprise over one-third of all congenital birth defects. High-throughput sequencing has recently led to the identification of many new causative disease genes and functional studies have clarified their mechanisms of action. We present recent findings in craniofacial genetics and discuss how this information together with developmental studies in animal models is helping to increase understanding of normal craniofacial development.
人类颅骨和面部的发育是一个高度协调且复杂的三维形态发生过程,涉及数百个基因,这些基因控制着具有多种胚胎起源的组织的协调模式形成、增殖和分化。由于发育异常而出现的颅面畸形(包括唇裂和/或腭裂、颅缝早闭和面部骨发育异常),占所有先天性出生缺陷的三分之一以上。高通量测序最近已导致鉴定出许多新的致病疾病基因,并且功能研究阐明了它们的作用机制。我们展示了颅面遗传学的最新发现,并讨论了这些信息以及动物模型中的发育研究如何有助于增进对正常颅面发育的理解。