The onset mechanism of primary Biliary cirrhosis

Shinji Shimoda

Research output: Chapter in Book/Report/Conference proceedingChapter


The principal homologous antigen of antimitochondrial antibodies is pyruvate dehydrogenase complex E2 component (PDC-E2) which is present in the mitochondrial inner membrane. The involvement of autoimmunity is considered due to the presence of both autoreactive CD4-positive T cells and CD8-positive T cells recognizing PDC-E2. Autoreactive NK cells are also involved in bile duct damage and in addition to acquired immunity, abnormalities in innate immunity are also very important regarding the onset of PBC. In addition to the mechanism of the bile duct epithelial cells presenting autoantigens, the pathology of chronic nonsuppurative destructive cholangitis (CNSDC) is maintained by means of antigen-presenting cells such as dendritic cells, macrophages, etc., which present autoantigens discharged from damaged bile duct epithelial cells.

Original languageEnglish
Title of host publicationAutoimmune Liver Diseases
Subtitle of host publicationPerspectives from Japan
PublisherSpringer Japan
Number of pages10
ISBN (Electronic)9784431547891
ISBN (Print)4431547886, 9784431547884
Publication statusPublished - Aug 1 2014

All Science Journal Classification (ASJC) codes

  • General Medicine


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