发育医学电子杂志 2023, Vol. 11 Issue (5): 384-388 DOI: 10.3969/j.issn.2095-5340.2023.05.010
结构畸形
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法尼醇X 受体影响胆道闭锁肝纤维化 机制的研究进展
于成皓 任红霞
(1. 山西医科大学 儿科医学系,山西 太原 030000;2. 山西 医科大学附属儿童医院 新生儿外科,山西 太原 030000)
Research progress on the mechanism of farnesoid X receptor affecting liver fibrosis in biliary atresia
Yu Chenghao, Ren Hongxia
摘要 胆道闭锁是一种罕见的导致新生儿及婴儿胆 汁淤积的先天性胆道系统疾病,以肝内外胆管进 行性炎症以及肝纤维化为特征,导致胆汁在胆道 内大量淤积,并最终引起肝硬化。如不及时治疗, 胆道闭锁患儿往往于出生后2 年内死于终末期肝 病[1-2] 。胆道闭锁早期诊断困难的特性与其高病死 率一直是困扰小儿外科医师的难题。转录因子法 尼醇X 受体(farnesoid X receptor,FXR)是一种表达 在人体肝脏,可以调节机体合成和利用胆汁酸的核 受体[3] 。转化生长因子(transforming growth factor, TGF)-β1 介导的Smad 信号通路是一种在发育胚 胎与成熟机体中均起着重要作用的细胞传导通路, 参与包括细胞生长、分化与凋亡在内的各种细胞 功能。诸多研究表明,FXR 与其介导的TGF-β1/ Smad 信号通路在调节胆道闭锁肝纤维化过程中 发挥重要作用[4-5] 。本文对FXR 直接激活或通过 TGF-β1/Smad 信号通路间接激活调节胆道闭锁中 肝纤维化进程进行分析与阐述,为揭示FXR 与胆 道闭锁肝纤维化的关系提供理论依据。
关键词:
胆道闭锁
肝纤维化
法尼醇衍生物X受体
收稿日期: 2022-08-30
出版日期: 2023-09-30
发布日期: 2023-09-27
期的出版日期: 2023-09-30
基金资助: 山西省卫生健康委员会科研课题(2022074);山西省儿童医院院内课题(201928)
通讯作者:
任红霞
E-mail: renhongxia100@sina.com
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