Fibrotic events in the progression of cholestatic liver disease

Cholestatic liver diseases including primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are associated with active hepatic fibrogenesis, which can ultimately lead to the development of cirrhosis. However, the exact relationship between the development of liver fibrosis and th...

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Detalhes bibliográficos
Autores: Wu, H. (Hanghang)|||/items/2f585fe8-bd2c-4260-97cd-e58447927de1, Chen, C. (Chaobo)|||/items/41de00bf-2d5f-4cf6-ac4b-8de3b5a73f1e, Ziani, S. (Siham)|||/items/110bfc8f-16a3-499f-b642-914d4a55a9d5, Nelson, L.J. (Leonard J.)|||/items/9714c156-8dd0-4cb1-a567-8201f996cbec, Avila, M.A. (Matías Antonio)|||/items/3ad9abbb-c18d-445b-86cf-cb76be15419f, Nevzorova, Y. (Yulia)|||/items/77dfb91c-326a-40ac-afe6-0c129e634d59, Cubero, F.J. (Francisco Javier)|||/items/0b630292-1741-4eec-b58e-9a843ca8198d
Formato: artículo
Fecha de publicación:2021
País:España
Recursos:Universidad de Navarra
Repositorio:Dadun. Depósito Académico Digital de la Universidad de Navarra
Idioma:inglés
OAI Identifier:oai:dadun.unav.edu:10171/115249
Acesso em linha:https://hdl.handle.net/10171/115249
Access Level:acceso abierto
Palavra-chave:Cholangiocytes
Cholestasis
Fibrosis
Hepatic stellate cells (HSCs)
Periductular fibroblasts
Descrição
Resumo:Cholestatic liver diseases including primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are associated with active hepatic fibrogenesis, which can ultimately lead to the development of cirrhosis. However, the exact relationship between the development of liver fibrosis and the progression of cholestatic liver disease remains elusive. Periductular fibroblasts located around the bile ducts seem biologically different from hepatic stellate cells (HSCs). The fibrotic events in these clinical conditions appear to be related to complex crosstalk between immune/inflammatory mechanisms, cytokine signalling, and perturbed homeostasis between cholangiocytes and mesenchymal cells. Several animal models including bile duct ligation (BDL) and the Mdr2-knockout mice have improved our understanding of mechanisms underlying chronic cholestasis. In the present review, we aim to elucidate the mechanisms of fibrosis in order to help to identify potential diagnostic and therapeutic targets.