• 1
    Walker JG, Doniach D, Roitt IM, Sherlock S. Serological tests in diagnosis of primary biliary cirrhosis. Lancet 1965; 1:827831.
  • 2
    Mackay IR. Primary biliary cirrhosis showing a high titer of autoantibody. N Engl J Med 1958; 258:185188.
  • 3
    Mouritsen S, Demant E, Permin H, Wiik A. High prevalence of antimitochondrial antibodies among patients with some well-defined connective tissue diseases. Clin Exp Immunol 1986; 66:6876.MEDLINE
  • 4
    Gilburd B, Ziporen L, Zharhary D, Blank M, Zurgil N, Scheinberg MA, Guedes LH, et al. Antimitochondrial (pyruvate dehydrogenase) antibodies in leprosy. J Clin Immunol 1994; 14:1419.MEDLINE
  • 5
    Zamfir O, Briaud I, Dubel L, Ballot E, Johanet C. Anti-pyruvate dehydrogenase autoantibodies in extrahepatic disorders. J Hepatol 1999; 31:964965.MEDLINE
  • 6
    Lang P, Klein R, Becker EW, Berg PA. Distribution of the PBC-specific-(M2) and the naturally-occurring mitochondrial antigen-(NOMAg) systems in plants. Clin Exp Immunol 1992; 90:509516.MEDLINE
  • 7
    Hazama H, Omagari K, Masuda J, Kinoshita H, Ohba K, Sakimura K, Matsuo I, et al. Serial changes in enzyme inhibitory antibody to pyruvate dehydrogenase complex during the course of primary biliary cirrhosis. J Clin Lab Anal 2000; 14:208213.MEDLINE
  • 8
    Gershwin ME, Mackay IR, Sturgess A, Coppel RL. Identification and specificity of a cDNA encoding the 70 KD mitochondrial antigen recognized in primary biliary cirrhosis. J Immunol 1987; 138:35253531.MEDLINE
  • 9
    Coppel RL, McNeilage LJ, Surh CD, Van de Water J, Spithill TW, Whittingham S, Gershwin ME. Primary structure of the human M2 mitochondrial autoantigen of primary biliary cirrhosis: dihydrolipoamide acetyltransferase. Proc Natl Acad Sci U S A 1988; 85:73177321.MEDLINE
  • 10
    Lau KS, Griffin TA, Hu CWC, Chuang DT. Conservation of primary structure in the lipoyl-bearing and dihydrolipoyl dehydrogenase binding domains of mammalian branched-chain alpha-keto acid dehydrogenase complex molecular cloning of human and bovine transacylase (E2) cDNAs. Biochemistry 1988; 27:19721981.MEDLINE
  • 11
    Surh CD, Danner DJ, Ahtab A, Coppel RL, Mackay IR, Dickson ER, Gershwin ME. Reactivity of primary biliary cirrhosis sera with a human fetal liver cDNA clone of branched-chain α-keto acid dehydrogenase dihydrolipoamide acyltransferase, 52 kD mitochondrial autoantigen. Hepatology 1989; 9:6368.MEDLINE
  • 12
    Moteki S, Leung PSC, Dickson ER, Van Thiel DH, Galperin C, Buch T, Alarcon-Segovia D, et al. Epitope mapping and reactivity of autoantibodies to the E2 component of 2-oxoglutarate dehydrogenase complex in primary biliary cirrhosis using recombinant 2-oxoglutarate dehydrogenase complex. Hepatology 1996; 23:436444.MEDLINE
  • 13
    Moteki S, Leung PSC, Coppel RL, Dickson ER, Kaplan MM, Munoz S, Gershwin ME. Use of a designer triple expression hybrid clone for three different lipoyl domains for the detection of antimitochondrial autoantibodies. Hepatology 1996; 24:97103.MEDLINE
  • 14
    Wiesner RH, LaRusso NF, Ludwig J, Dickson ER. Comparison of the clinicopathologic features in primary sclerosing cholangitis and primary biliary cirrhosis. Gastroenterology 1985; 88:108114.MEDLINE
  • 15
    Studier FW. Use of bacteriophage T7 lysozyme to improve an inducible T7 expression system. J Mol Biol 1991; 219:3744.MEDLINE
  • 16
    Ali G, Wasco W, Cai X, Szabo P, Sheu KF, Cooper AJ, Gaston SM, et al. Isolation, characterization, and mapping of gene encoding dihydrolipoil succinyltransferase (E2k) of human alpha-ketoglutarate dehydrogenase complex. Somat Cell Mol Genet 1994; 20:99105.MEDLINE
  • 17
    Laemmli UK. Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature 1970; 277:680685.
  • 18
    Towbin H, Staehelin T, Gordon J. Electrophoretic transfer of proteins from polyacrylamide gels to nitrocellulose sheets: procedure and some applications. Proc Natl Acad Sci U S A 1979; 76:43504354.MEDLINE
  • 19
    Miyakawa H, Kawaguchi N, Abe K, Kitazawa E, Kikuchi K, Fujikawa H, Matsushita M, et al. Serial changes of serum anti-M2 proteins in patients with primary biliary cirrhosis: a follow-up study by immunoblotting. Hepatol Res 1999; 13:143152.
  • 20
    Nakanuma Y, Tsuneyama K, Gershwin ME, Yososhima M. Pathology and immunopathology of primary biliary cirrhosis with emphasis on bile duct lesions: recent progress. Semin Liver Dis 1995; 15:313328.MEDLINE
  • 21
    Miyakawa H, Kawaguchi N, Kikuchi K, Kitazawa E, Kawashima Y, Yajima R, Itoh Y. False positive reaction in ELISA for IgM class anti-M2 antibody and its prevention. Hepatol Res 2001, in press.
  • 22
    Van de Water J, Cooper A, Surh CD, Coppel R, Danner D, Ansari A, Dickson R, et al. Detection of autoantibodies to recombinant mitochondrial proteins in patients with primary biliary cirrhosis. N Engl J Med 1989; 320:13771380.MEDLINE
  • 23
    Miyakawa H, Abe K, Kitazawa E, Kikuchi K, Fujikawa H, Matsushita M, Kawaguchi N, et al. Detection of anti-branched chain 2-oxo acid dehydrogenase complex (BCOADC)-E2 antibody in primary biliary cirrhosis by ELISA using recombinant fusion protein. Autoimmunity 1999; 30:1120.MEDLINE
  • 24
    Kikuchi K. Immunoreactivity of recombinant human oxo-glutarate dehydrogenase complex (OGDC)-E2 protein in primary biliary cirrhosis. Hepatol Res 2000; 16:211223.
  • 25
    Rosenberg AH, Lade BN, Chui D, Lin S-W, Dunn JJ, Studier FW. Vectors for selective expression of cloned DNAs by T7 RNA polymerase. Gene 1987; 56:125135.MEDLINE
  • 26
    Iwayama T, Leung PSC, Coppel RL, Roche TE, Patel MS, Mizushima Y, Nakagawa T, et al. Specific reactivity and mapping of recombinant PDC-E1a in primary biliary cirrhosis. J Autoimmunity 1991; 4:769778.
  • 27
    Surh CD, Coppel R, Gershwin ME. Structral requirement for autoreactivity on human pyruvate dehydrogenase-E2, the major autoantigen of primary biliary cirrhosis. Implication for a conformational autoepitope. J Immunol 1990; 144:33673374.MEDLINE
  • 28
    Miyakawa H, Kawaguchi N, Kikuchi K, Fujikawa H, Kitazawa E, Matsushita M, Abe K, et al. Combination assays for IgG class and IgM class anti-pyruvate dehydrogenase complex (PDC)-E2 by ELISA using recombinant autoantigen to diagnose primary biliary cirrhosis. Hepatol Res 1998; 12:140150.
  • 29
    Mutimer DJ, Fussey SP, Yeaman SJ, Lelly PJ, James OF, Bassendine MF. Frequency of IgG and IgM autoantibodies to four specific M2 mitochondrial autoantigens in primary biliary cirrhosis. Hepatology 1989; 10:403407.MEDLINE
  • 30
    Brünner G, Klainge O. A cholangitis with antinuclear antibodies (immuno-cholangitis) resembling chronic non-suppurative destructive cholangitis. Dtsch Med Wochenschr 1987; 112:14541458.MEDLINE
  • 31
    Ben-Ari Z, Dhillon AP, Sherlock S. Autoimmune cholangiopathy: part of the spectrum of autoimmune chronic active hepatitis. Hepatology 1993; 18:1015.MEDLINE
  • 32
    Colombata LA, Alvarez F, Cote J, Huet PM. Autoimmune cholangiopathy: the result of consecutive primary biliary cirrhosis and autoimmune hepatitis? Gastroenterology 1994; 107:18391843.MEDLINE
  • 33
    Lacerda MA, Ludwig J, Dickson ER, Jorgensen RA, Lindor MD. Antimitochondrial antibody-negative primary biliary cirrhosis. Am J Gastroenterol 1990; 90:247249.
  • 34
    Sherlock DS. Autoimmune cholangitis: a unique entity? Mayo Clin Proc 1998; 73:184190.MEDLINE