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.
Keywords: Break of tolerance against PDC-E2, antimitochondrial autoantibodies, autoimmune cholangitis,
autoreactive T cells, cytotoxic CD8+ T cells, defective treg cells, cholestasis, AE2 deficiency, deficient bicarbonate secretion.
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