Detection of Autoantibodies to Recombinant Mitochondrial Proteins in Patients with Primary Biliary Cirrhosis

Abstract

Primary biliary cirrhosis is characterized by the presence of autoantibodies to mitochondria with specific reactivity to proteins of 74 and 52 kilodaltons (kd). The 74-kd mitochondrial protein is the E2 component — dihydrolipoamide acetyltransferase — of the pyruvate dehydrogenase complex, and the 52-kd protein is the equivalent E2 component — dihydrolipoamide acyltransferase — of the branched-chain α-keto acid dehydrogenase complex. Current methods for the detection of antibodies to these proteins lack specificity or sensitivity, or they are time-consuming and not readily available.