Fig. 2
From: Animal models of primary biliary cholangitis: status and challenges
Disease characteristics in genetically modified and inducible animal models of PBC. Establishment of genetically modified models is based on genetic factors or Treg dysfunction. Chemical and infectious agents can also induce PBC development in mice. Pyruvate dehydrogenase complex (PDC)-E2 mimics or modified PDC-E2 can induce a loss of autoantigen tolerance and multilineage immune dysregulation. The main pathogenic mechanisms of PBC are between bile acid (BAH)-induced epithelial damage and autoimmune attack of bile duct epithelial cells (BECs), eventually progressing to liver fibrosis and cirrhosis