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哺乳动物的成纤维细胞生长因子家族由18种分泌蛋白组成,它们与酪氨酸激酶组成的4种FGF受体作用以传递信号。FGF与其信号受体的相互作用受到蛋白质、蛋白多糖辅因子以及细胞外结合蛋白的调节。激活的FGFRs使特定的氨基酸残基磷酸化,从而调节与细胞内接头蛋白的相互作用以及RAS-MAPK,PI3K-AKT,PLCγ、STAT等胞内信号通路。FGF家族成员在胚胎发育的最初阶段即开始发挥作用,在器官形成阶段维持祖细胞并调节祖细胞的生长、分化、存活等。FGFs几乎出现在所有组织及器官中,本文主要阐述FGF信号通路的异常活动与颅面部发育缺陷之间的关系。
The mammalian family of fibroblast growth factors consists of 18 secreted proteins that function with four FGF receptors composed of tyrosine kinases to transmit signals. The interaction of FGF with its signaling receptors is regulated by proteins, proteoglycan cofactors, and extracellular binding proteins. Activated FGFRs phosphorylate specific amino acid residues, thereby regulating intracellular adapter protein interactions and intracellular signaling pathways such as RAS-MAPK, PI3K-AKT, PLCγ, STAT and the like. FGF family members begin to play an initial role in embryonic development, and maintain progenitor cells and regulate the growth, differentiation and survival of progenitor cells during the organogenesis stage. FGFs occur in almost all tissues and organs, this article mainly describes the relationship between abnormal FGF signaling and craniofacial developmental defects.