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English: Fig. 1. The pathogenesis of PBC. 1. On a permissive genetic background, exposure to a microbial or environment similar to PDC-E2 will induce an immune response. 2. Attacked by cytotoxic T cells and loss of AE2 function will lead to the apoptosis of BEC, and PDC-E2 epitope immunologically intact with an apoptotic bleb due to lack of covalent binding of glutathione. 3. Recognition of AMA and the formation of immune complex further activates the immune system, leading to persistent and widespread BEC damage. 4. The innate and adaptive immunity involve in the further propagates BEC injury and eventually lead to the deterioration of the disease.