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

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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.