Primary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by serum antimitochondrial antibody mainly reacting with inner lipoyl domain of E2 subunit of pyruvate dehydrogenase (PDC-E2). Lipoyl domain of PDC-E2 has sequence homologies with biotinylated site of biotin enzymes. This biotin-related protein and the C355.1 reactive antigen were surveyed by Western blotting in gallbladder bile samples from 27 patients with PBC and from 23 patients with other hepatobiliary disease. Biotin-related proteins of 70 and 72 kDa were detected in 89% of PBC samples, whereas it was infrequently detected in 48% of control samples ( P<0.01). C355.1 reactive antigen of 64 kDa was detected in 22% of PBC samples and 8.7% of control samples ( P>0.01). This biotin-related proteins of 70 and 72 kDa may refer to the pathogenesis of PBC.