Dubey Aditi, Saint-Jeannet Jean-Pierre
Department of Basic Science & Craniofacial Biology, College of Dentistry, New York University, New York, USA.
Curr Pathobiol Rep. 2017 Mar;5(1):79-92. doi: 10.1007/s40139-017-0128-8. Epub 2017 Jan 24.
Craniofacial disorders are among the most common human birth defects and present an enormous health care and social burden. The development of animal models has been instrumental to investigate fundamental questions in craniofacial biology and this knowledge is critical to understand the etiology and pathogenesis of these disorders.
The vast majority of craniofacial disorders arise from abnormal development of the neural crest, a multipotent and migratory cell population. Therefore, defining the pathogenesis of these conditions starts with a deep understanding of the mechanisms that preside over neural crest formation and its role in craniofacial development.
This review discusses several studies using embryos to model human craniofacial conditions, and emphasizes the strength of this system to inform important biological processes as they relate to human craniofacial development and disease.
颅面疾病是最常见的人类出生缺陷之一,带来了巨大的医疗和社会负担。动物模型的发展对于研究颅面生物学的基本问题至关重要,而这些知识对于理解这些疾病的病因和发病机制至关重要。
绝大多数颅面疾病源于神经嵴的异常发育,神经嵴是一种多能迁移细胞群体。因此,要确定这些疾病的发病机制,首先要深入了解主导神经嵴形成的机制及其在颅面发育中的作用。
本综述讨论了几项利用胚胎模拟人类颅面疾病的研究,并强调了该系统在揭示与人类颅面发育和疾病相关的重要生物学过程方面的优势。