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Cholangiocyte pathobiology.
Nature Reviews Gastroenterology & Hepatology ( IF 45.9 ) Pub Date : 2019-05-01 , DOI: 10.1038/s41575-019-0125-y
Jesus M Banales 1 , Robert C Huebert 2 , Tom Karlsen 3 , Mario Strazzabosco 4 , Nicholas F LaRusso 2 , Gregory J Gores 2
Affiliation  

Cholangiocytes, the epithelial cells lining the intrahepatic and extrahepatic bile ducts, are highly specialized cells residing in a complex anatomic niche where they participate in bile production and homeostasis. Cholangiocytes are damaged in a variety of human diseases termed cholangiopathies, often causing advanced liver failure. The regulation of cholangiocyte transport properties is increasingly understood, as is their anatomical and functional heterogeneity along the biliary tract. Furthermore, cholangiocytes are pivotal in liver regeneration, especially when hepatocyte regeneration is compromised. The role of cholangiocytes in innate and adaptive immune responses, a critical subject relevant to immune-mediated cholangiopathies, is also emerging. Finally, reactive ductular cells are present in many cholestatic and other liver diseases. In chronic disease states, this repair response contributes to liver inflammation, fibrosis and carcinogenesis and is a subject of intense investigation. This Review highlights advances in cholangiocyte research, especially their role in development and liver regeneration, their functional and biochemical heterogeneity, their activation and involvement in inflammation and fibrosis and their engagement with the immune system. We aim to focus further attention on cholangiocyte pathobiology and the search for new disease-modifying therapies targeting the cholangiopathies.

中文翻译:

胆管细胞病理生物学。

胆管细胞是位于肝内和肝外胆管内的上皮细胞,是高度复杂的细胞,位于复杂的解剖位中,参与胆汁的产生和体内稳态。胆管细胞在称为胆管病的多种人类疾病中受损,通常会导致晚期肝衰竭。人们越来越了解胆管细胞转运特性的调节,以及它们沿胆道的解剖学和功能异质性。此外,胆管细胞在肝再生中至关重要,尤其是在肝细胞再生受到损害时。胆管细胞在先天性和适应性免疫反应中的作用也正在兴起,这是与免疫介导的胆管病相关的关键主题。最后,反应性导管细胞存在于许多胆汁淤积和其他肝脏疾病中。在慢性疾病状态下,这种修复反应导致肝脏炎症,纤维化和癌变,并且是深入研究的主题。这篇综述重点介绍了胆管细胞研究的进展,特别是它们在发育和肝脏再生中的作用,它们的功能和生化异质性,它们在炎症和纤维化中的激活和参与以及它们与免疫系统的结合。我们的目标是将更多的注意力集中在胆管细胞病理生物学和寻找针对胆管疾病的新的疾病缓解疗法上。它们的功能和生化异质性,它们的活化和参与炎症和纤维化以及它们与免疫系统的结合。我们的目标是将更多的注意力集中在胆管细胞病理生物学和寻找针对胆管疾病的新的疾病缓解疗法上。它们的功能和生化异质性,它们的活化和参与炎症和纤维化以及它们与免疫系统的结合。我们的目标是将更多的注意力集中在胆管细胞病理生物学和寻找针对胆管疾病的新的疾病缓解疗法上。
更新日期:2019-03-09
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