背景:已经开发了各种模仿颅骨融合的动物模型,使用突变斑马鱼和小鼠。本文的目的是回顾不同的综合征性颅骨融合动物模型,并分析它们为我们对这些疾病的病理生理学的理解提供了哪些见解。
方法:综述了颅骨融合动物模型的相关文献。
结果:尽管很少发表关于斑马鱼颅骨融合的研究,该模型在研究脊椎动物保守的缝合线形成机制方面似乎很有用.相反,已经为最常见的综合征性颅骨滑脱症生成了几种小鼠模型,与FGFR1,FGFR2,FGFR3和TWIST基因以及MSX2,EFFNA,GLI3、FREM1、FGF3/4基因。小鼠模型也已用于测试恢复颅面生长的药物治疗。
结论:近几十年来已经开发了几种斑马鱼和小鼠模型。这些动物模型有助于我们对正常和病理性颅面生长的理解。模拟颅骨滑脱症的小鼠模型可以容易地用于筛选作为治疗候选物的药物。
BACKGROUND: Various animal models mimicking craniosynostosis have been developed, using mutant zebrafish and mouse. The aim of this paper is to review the different animal models for syndromic craniosynostosis and analyze what insights they have provided in our understanding of the pathophysiology of these conditions.
METHODS: The relevant literature for animal models of craniosynostosis was reviewed.
RESULTS: Although few studies on craniosynostosis using zebrafish were published, this model appears useful in studying the suture formation mechanisms conserved across vertebrates. Conversely, several mouse models have been generated for the most common syndromic craniosynostoses, associated with mutations in FGFR1, FGFR2, FGFR3 and TWIST genes and also in MSX2, EFFNA, GLI3, FREM1, FGF3/4 genes. The mouse models have also been used to test pharmacological treatments to restore craniofacial growth.
CONCLUSIONS: Several zebrafish and mouse models have been developed in recent decades. These animal models have been helpful for our understanding of normal and pathological craniofacial growth. Mouse models mimicking craniosynostoses can be easily used for the screening of drugs as therapeutic candidates.