Faculty of Dentistry, McGill University, Montreal, QC, Canada.
Division of Experimental Medicine, Department of Medicine, McGill University, Montreal, QC, Canada.
Oral Dis. 2018 Mar;24(1-2):78-83. doi: 10.1111/odi.12758.
Craniofacial development is a delicate process that involves complex interactions among cells of multiple developmental origins, their migration, proliferation, and differentiation. Tissue morphogenesis of the craniofacial skeleton depends on genetic and environmental factors, and on specific signaling pathways, which are still not well understood. Developmental defects of the midface caused by the absence, delays, or premature fusion of nasal and maxillary prominences vary in severity; leading to clefts, hypoplasias, and midline expansion. In the current review, we focus on the importance of the chondrocranium in craniofacial growth and how its impaired development leads to midface hypoplasia. More importantly, we reported how Matrix Gla protein (MGP), a potent inhibitor of extracellular matrix mineralization, facilitates midface development by preventing ectopic calcification of the nasal septum. In fact, MGP may act as a common link in multiple developmental pathologies all showing midface hypoplasia caused by abnormal cartilage calcification. This brief review discusses the gap in knowledge in the field, raises pertinent questions, which remain unanswered, and sheds light on the future research directions.
颅面发育是一个复杂的过程，涉及到多个发育起源的细胞之间的复杂相互作用，包括它们的迁移、增殖和分化。颅面骨骼的组织形态发生依赖于遗传和环境因素，以及特定的信号通路，但这些仍然没有得到很好的理解。由于鼻和上颌突的缺失、延迟或过早融合导致的中面部发育缺陷，严重程度不一；导致裂隙、发育不良和中线扩张。在本次综述中，我们重点关注软骨颅的重要性及其发育障碍如何导致中面部发育不良。更重要的是，我们报告了基质 Gla 蛋白（MGP），一种细胞外基质矿化的有效抑制剂，如何通过防止鼻中隔的异位钙化来促进中面部的发育。事实上，MGP 可能在多种表现出中面部发育不良的发育异常疾病中起共同作用，这些疾病都是由异常软骨钙化引起的。本文简要综述了该领域的知识空白，提出了一些悬而未决的问题，并为未来的研究方向提供了启示。
