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
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 – 1 April 1995 – Primary biliary cirrhosis (PBC) is a chronic autoimmune liver disease characterized histologically by non‐suppurative 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).