Kudira Ramesh, Yang Zi F, Osuji Immaculeta, Karns Rebekah, Bariya Priya, Pfuhler Liva, Mullen Mary, Taylor Amy, Ji Hong, Lages Celine S, Yang Vom Hofe Annika, Shi Tiffany, Pasula Srikar, Wayman Joseph A, Bernieh Anas, Zhang Wujuan, Chougnet Claire A, Hildeman David, Tiao Gregory M, Huppert Stacey S, Subramanian Sajay, Salomonis Nathan, Miraldi Emily, Miethke Alexander Gerhard
bioRxiv. 2024 May 21:2024.05.17.594680. doi: 10.1101/2024.05.17.594680.
Fibrosing cholangiopathies, including biliary atresia and primary sclerosing cholangitis, involve immune-mediated bile duct epithelial injury and hepatic bile acid (BA) retention (cholestasis). Regulatory T-cells (Tregs) can prevent auto-reactive lymphocyte activation, yet the effects of BA on this CD4 lymphocyte subset are unknown. Gene regulatory networks for hepatic CD4 lymphocytes in a murine cholestasis model revealed Tregs are polarized to Th17 during cholestasis. Following bile duct ligation, Stat3 deletion in CD4 lymphocytes preserved hepatic Treg responses. While pharmacological reduction of hepatic BA in MDR2-/- mice prompted Treg expansion and diminished liver injury, this improvement subsided with Treg depletion. A cluster of patients diagnosed with biliary atresia showed both increased hepatic Treg responses and improved 2-year native liver survival, supporting that Tregs might protect against neonatal bile duct obstruction. Together, these findings suggest liver BA determine Treg function and should be considered as a therapeutic target to restore protective hepatic immune responses.
纤维化性胆管病,包括胆道闭锁和原发性硬化性胆管炎,涉及免疫介导的胆管上皮损伤和肝胆汁酸(BA)潴留(胆汁淤积)。调节性T细胞(Tregs)可防止自身反应性淋巴细胞激活,但BA对该CD4淋巴细胞亚群的影响尚不清楚。在小鼠胆汁淤积模型中,肝CD4淋巴细胞的基因调控网络显示,在胆汁淤积期间Tregs向Th17极化。胆管结扎后,CD4淋巴细胞中的Stat3缺失可保留肝Treg反应。虽然MDR2-/-小鼠肝脏BA的药理学降低促使Treg扩增并减轻肝损伤,但随着Treg耗竭,这种改善减弱。一组被诊断为胆道闭锁的患者显示肝Treg反应增加且2年自体肝存活率提高,支持Tregs可能预防新生儿胆管梗阻。总之,这些发现表明肝脏BA决定Treg功能,应被视为恢复保护性肝免疫反应的治疗靶点。