Abnormal expression of the E2 component of the pyruvate dehydrogenase complex on the luminal surface of biliary epithelium occurs before major histocompatibility complex class II and BB1/B7 expression
✍ Scribed by Koichi Tsuneyama; Judy van de Water; Patrick S. C. Leung; Sanghoon Cha; Yasuni Nakanuma; Marshall Kaplan; Ronald de Lellis; Ross Coppel; Aftab Ansari; M. Eric Gershwin
- Publisher
- John Wiley and Sons
- Year
- 1995
- Tongue
- English
- Weight
- 797 KB
- Volume
- 21
- Category
- Article
- ISSN
- 0270-9139
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✦ Synopsis
Primary biliary cirrhosis (PBC) is a chronic autoimmune liver disease characterized histologically by nonsuppurative destructive cholangitis. Sera from patients with PBC react with a series of intramitochondrial enzymes with the immunodominant response directed against the E2 component of the pyruvate dehydrogenase complex (PDC-E2). Recently, using tissue sections of late-stage PBC, we showed that there is increased expression in biliary epithelial cells of patients with PDC-E2 or a molecule cross-reactive with PDC-E2. Previous work has shown that biliary epithelial cells of patients with PBC express an increased amount of class 11. To address the sequence of events in the evolution of PBC, we have focused our attention in this study on early biliary epithelial lesions. In particular, we have studied the liver of 22 female patients with PBC that was diagnosed as either stage I or stage I1 using both a mouse monoclonal antibody that has reactivity similar to human autoantibodies as well as a human Fab combinatorial prepared from the lymph node of a PBC patient. Tissues were simultaneously stained using antibodies to PDC-EZ, class 11, and BByB7. As a positive control, tissues from late-stage PBC were studied concurrently. By determining the order of expression among the three molecules, PDC-E2, class 11, and BBUI37, we report that the expression of PDC-E2 or a PDC-E2-like molecule on