Primary Biliary Cirrhosis - Specific Anti-mitochondrial Antibodies

Summary

Primary biliary cirrhosis is a chronic liver disease, characterized by the destruction of the epithelialcells of the sublobular, interlobular and septal bile ducts and with the development of cirrhosis.The presence of anti-mitochondrial antibodies against the subunits of mitochondrial2-oxoacids dehydrogenases is characteristic for patients with primary biliary cirrhosis. The aimof this work was to study the effect of anti-mitochondrial antibodies upon activity of the isolatedmitochondrial pyruvate dehydrogenase complex after the incubation with serum from patientswith primary biliary cirrhosis. Group of patients and methods. The activity of the purified bovinepyruvate dehydrogenase complex was studied spectrophotometrically in presence of the serum (1: 1000) from five patients with primary biliary cirrhosis and from ten disease free controls. Results.The activity of the pyruvate dehydrogenase was decreased after incubation with the serumfrom patients with primary biliary cirrhosis. No similar inhibitory effect was found after incubationwith serum from controls. Discussion. The inhibitory effect of the anti-mitochondrial antibodiesupon activity of pyruvate dehydrogenase may broaden the spectrum of diagnostic methods inpatients with primary biliary cirrhosis. Further investigations are necessary to assess the possibleapplication of this method for monitoring of changes during the course of the disease and forassignation of the disease prognosis.

Key words:
Primary biliary cirrhosis - Anti-mitochondrial antibodies - Pyruvate dehydrogenasecomplex