Mouse models of primary biliary cirrhosis

Current Pharmaceutical Design
Axel R Concepcion, Juan F Medina

Abstract

Primary biliary cirrhosis (PBC) is a chronic and progressive cholestatic liver disease of unknown etiopathogenesis that mainly affects middle-aged women. Patients show non-suppurative cholangitis with damage and destruction of small- and medium-sized intrahepatic bile ducts. Characteristically, the disease is strongly associated with autoimmune phenomena such as the appearance of serum antimitochondrial autoantibodies (AMA) and portal infiltrates with autoreactive T cells which recognize the inner lipoyl domain of the E2 component of the pyruvate dehydrogenase complex (PDC-E2). Here we review the major characteristics of a series of inducible and genetically modified animal models of PBC and analyze their similarities and differences with PBC features in humans.

Citations

May 13, 2017·Frontiers in Immunology·Nicola L DinyDaniela Čiháková
Mar 10, 2020·Endocrine, Metabolic & Immune Disorders Drug Targets·Thea MagroneEmilio Jirillo
Sep 9, 2021·The Journal of Experimental Medicine·Juliette PailletJonathan G Pol

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