Axel R. Concepcion and Juan F. Medina Pages 2401 - 2413 ( 13 )
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.
Break of tolerance against PDC-E2, antimitochondrial autoantibodies, autoimmune cholangitis, autoreactive T cells, cytotoxic CD8<sup>+</sup> T cells, defective treg cells, cholestasis, AE2 deficiency, deficient bicarbonate secretion.
, Division of Gene Therapy and Hepatology, CIMA, Clinic and School of Medicine University of Navarra, and Ciberehd, Pamplona, Spain.