Abstract：
Fibroblast growth factor 9 (FGF9) was first identified during a screen for factors acting on cells of the central nervous system (CNS). Research over the subsequent two decades has revealed this protein to be a critically important and elegantly regulated growth factor. A hallmark control feature is reciprocal compartmentalization, particularly during development, with epithelium as a dominant source and mesenchyme a prime target. This mesenchyme selectivity is accomplished by the high affinity of FGF9 to the IIIc isoforms of FGFR1, 2, and 3. FGF9 is expressed widely in the embryo, including the developing heart and lungs, and more selectively in the adult, including the CNS and kidneys. Global Fgf9-null mice die shortly after birth due to respiratory failure from hypoplastic lungs. As well, their hearts are dilated and poorly vascularized, the skeleton is small, the intestine is shortened, and male-to-female sex reversal can be found. Conditional Fgf9-null mice have revealed CNS phenotypes, including ataxia and epilepsy. In humans, FGF9 variants have been found to underlie multiple synostoses syndrome 3, a syndrome characterized by multiple joint fusions. Aberrant FGF9 signaling has also been implicated in differences of sex development and cancer, whereas vascular stabilizing effects of FGF9 could benefit chronic diseases. This primer reviews the attributes of this vital growth factor.
摘要：
在筛选作用于中枢神经系统（CNS）细胞的因子期间，首先鉴定了成纤维细胞生长因子9（FGF9）。在随后的二十年中的研究表明，这种蛋白质是一种至关重要且调节优雅的生长因子。一个标志控制功能是相互划分，特别是在开发过程中，以上皮为主要来源，间充质为主要目标。这种间充质选择性是通过FGF9对FGFR1、2和3的IIIc同工型的高亲和力来实现的。FGF9在胚胎中广泛表达，包括发育中的心脏和肺，在成年人身上更有选择性，包括中枢神经系统和肾脏。全球Fgf9无效小鼠在出生后不久由于发育不良肺引起的呼吸衰竭而死亡。同样,他们的心脏扩张，血管化不良，骨架很小，肠道缩短了，可以发现男性对女性的性别逆转。有条件的Fgf9-null小鼠已经揭示了CNS表型，包括共济失调和癫痫。在人类中,已发现FGF9变体是多发性骨膜综合征3的基础，该综合征以多个关节融合为特征。FGF9信号异常也与性发育和癌症的差异有关。而FGF9的血管稳定作用可使慢性疾病受益。本入门回顾了这种重要生长因子的属性。
