期刊
NATURE REVIEWS GASTROENTEROLOGY & HEPATOLOGY
卷 16, 期 5, 页码 269-281出版社
NATURE PORTFOLIO
DOI: 10.1038/s41575-019-0125-y
关键词
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资金
- Spanish Ministries of Economy and Competitiveness [FIS PI15/01132, FIS PI18/01075, CON14/00129]
- 'Fondo Europeo de Desarrollo Regional' (FEDER)
- ISCIII (CIBERehd), Spain
- BIOEF (Basque Foundation for Innovation and Health Research)
- Department of Health of the Basque Country [2017111010]
- Scientific Foundation of the Spanish Association Against Cancer (AECC)
- US National Institutes of Health [DK079005, DK096096, DK34989, DK63947, DK057993, DK084567, DK24031, DK100575, DK113339]
- Carlos Family Foundation
- Mayo Clinic
- PSC Partners Seeking a Cure
- Silvio O. Conte Digestive Diseases Research Core Centers
- EiTB Maratoia [BIO15/CA/016/BD]
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.
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