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Fibrotic Events in the Progression of Cholestatic Liver Disease Wu, Hanghang Wu, Hanghang Chen, Chaobo Ziani, Siham Nelson, Leonard J Ávila, Matías A Nevzorova, Yulia Cubero Palero, Francisco Javier Cholangiocytes; ; ; ; Hepatic stellate cells (HSCs) Periductular fibroblasts Cholestasis Fibrosis Ciencias Biomédicas Biología Medicina Inmunología 24 Ciencias de la Vida 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. 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 Mdr2knockout 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. Ministerio de Economía, Comercio y Empresa (España) Deutsche Forschungsgemeinschaft Depto. de Inmunología, Oftalmología y ORL Fac. de Medicina TRUE pub 2024-03-08T12:45:19Z 2024-03-08T12:45:19Z 2021 journal article VoR https://hdl.handle.net/20.500.14352/102069 2073-4409 10.3390/cells10051107 eng Retos SAF2016-78711 info:eu-repo/grantAgreement/AEI/Plan Estatal de Investigación Científica y Técnica y de Innovación 2013-2016/SAF2017-87919-R/ES/INTERDEPENDENCIA DEL ESTRES OXIDATIVO Y LA INFLAMACION DURANTE LA ENFERMEDAD HEPATICA CRONICA: UNA NUEVA HERRAMIENTA TERANOSTICA/ PID2019-104878RB-100AEI/10.13039/501100011033 EXOHEP-CM S2017/BMD-3727 NanoLiver-CM Y2018/NMT-4949 ERAB Ref. EA 18/14 AMMF 2018/117 UCM-25-2019 SFB/TRR57/P04 SFB 1382-403224013/A02 DFG NE 2128/2-1 Wu H, Chen C, Ziani S, Nelson LJ, Ávila MA, Nevzorova YA, Cubero FJ. Fibrotic Events in the Progression of Cholestatic Liver Disease. Cells. 2021 May 5;10(5):1107. doi: 10.3390/cells10051107. PMID: 34062960; PMCID: PMC8147992. Attribution 4.0 International http://creativecommons.org/licenses/by/4.0/ open access application/pdf MDPI