title: Mouse models of primary biliary cirrhosis.
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.
authors.forename: Axel R
authors.initials: AR
authors.lastname: Concepcion
authors.affiliation: Division of Gene Therapy and Hepatology, CIMA, Clinic and School of Medicine University of Navarra, and Ciberehd, Pamplona, Spain. jfmedina@unav.es.
authors.forename: Juan F
authors.initials: JF
authors.lastname: Medina
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000276:immunology; D004195:Disease Models, Animal; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D051379:Mice
chemical_list: D001323:Autoantibodies