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
Open accessAbstract
Five different target mitochondrial autoantigens recognized by sera from patients with primary biliary cirrhosis (PBC) have been identified as subunits of the following 2-oxo acid dehydrogenase complexes: the pyruvate dehydrogenase complex (PDC), the branched chain 2-oxo acid dehydrogenase complex (BCOADC), and the 2-oxoglutarate dehydrogenase complex (OGDC). Unlike the E2 subunits of PDC (PDC-E2) and BCOADC (BCOADC-E2), the E2 subunits of OGDC (OGDC-E2) reactivity of PBC sera and the reactive epitope of OGDC-D2 have not hitherto been studied in detail. In this report, we took advantage of a recombinant fusion protein for OGDC-E2 to address these issues. Eighty of 268 (29.9%) PBC patient sera but none of the 45 controls reacted with recombinant OGDC-E2. The recombinant OGDC-E2 was judged to express the immunodominant epitope, because when sera from patients with PBC were preabsorbed with the recombinant fusion protein, such sera were depleted of reactivity against 48 kD OGDC-E2 when probed on beef heart mitochondria (BHM) but retained reactivity toward PDC-E2 and/or BCOADC-E2. Furthermore, affinity-purified PBC sera against recombinant OGDC-E2 reacted only with native OGDC-E2 and not with any other enzyme components of the 2-oxo acid dehydrogenase complex. Antimitochondrial autoantibodies (AMA) against OGDC-E2 included immunoglobulin (Ig)G2, IgG3 and IgM and the relative titers were as follows: IgG2 > IgG3 > IgM. Finally, using overlapping recombinant polypeptides, it was determined that a minimum of 81 amino acids (residues 67-147) corresponding to the lipoyl domain of OGDC-E2 are necessary for reactivity, suggesting that a conformational autoepitope is recognized by AMA. These data suggest that each of the 2-oxo acid dehydrogenase enzymes has distinct antigenicity despite their similarities in structure and function. The availability of recombinant OGDC-E2 autoantigen will allow the design of additional studies to further our understanding of the role of mitochondrial autoantigens in the pathogenesis of PBC.
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AMA is a highly disease-specific autoantibody that targets lipoic acid on the 2-oxo-acid dehydrogenase complexes of the inner mitochondrial membrane.
“AMA is a highly disease-specific autoantibody (8) that targets the lipoic acid present on the 2-oxo-acid dehydrogenase complexes located on the inner mitochondrial membrane.(9)”
From Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases (2019) · cites this paper as [9]
Citation (9) is attached to the targeting and location clause, not the disease-specificity clause cited to (8). The Abstract identifies E2 subunits of 2-oxo-acid dehydrogenase complexes as AMA targets, and the epitope-mapping discussion places a recognized epitope in OGDC-E2’s lipoic-acid-binding region. It does not establish that AMA targets lipoic acid itself or that the complexes are located on the inner mitochondrial membrane; the discussion also says lipoic acid’s presence did not affect antibody binding to BCOADC-E2. Page numbers are unavailable in our copy. (LLM, conf=0.86)
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Cites
Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4
LAEMMLI, U. K. (1970) · Nature · doi:10.1038/227680a0 · Cited by 249,961 · see citing works
Towbin, H., Staehelin, T., & Gordon, J. (1979) · Proceedings of the National Academy of Sciences of the United States of America · doi:10.1073/pnas.76.9.4350 · Cited by 51,421 · see citing works
INTRACELLULAR DISTRIBUTION OF ENZYMES
Schneider, W., & Hogeboom, G. H. (1950) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)56449-8 · Cited by 1,357 · see citing works
Schwab, M. (2011) · Springer eBooks · doi:10.1007/978-3-642-16483-5_4730 · Cited by 1,241 · see citing works
Gershwin, M. E., Mackay, I. R., Sturgess, A., & Coppel, R. L. (1987) · The Journal of Immunology · doi:10.4049/jimmunol.138.10.3525 · Cited by 557 · see citing works
Self-citationSEROLOGICAL TESTS IN DIAGNOSIS OF PRIMARY BILIARY CIRRHOSIS
WALKER, J. (1965) · The Lancet · doi:10.1016/s0140-6736(65)91372-3 · Cited by 507 · see citing works
PRIMARY BILIARY CIRRHOSIS: IDENTIFICATION OF TWO MAJOR M2 MITOCHONDRIAL AUTOANTIGENS
Yeaman, S. J., Danner, D. J., Mutimer, D., Fussey, S. P. M., James, O., & Bassendine, M. F. (1988) · The Lancet · doi:10.1016/s0140-6736(88)91894-6 · Cited by 292 · see citing works
de Water, J. V., Gershwin, M. E., Leung, P. S. C., Ansari, A. A., & Coppel, R. L. (1988) · The Journal of Experimental Medicine · doi:10.1084/jem.167.6.1791 · Cited by 279 · see citing works
Self-citationIdentification and analysis of the major M2 autoantigens in primary biliary cirrhosis.
Fussey, S. P., Guest, J. R., James, O. F., Bassendine, M. F., & Yeaman, S. J. (1988) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.85.22.8654 · Cited by 273 · see citing works
Coppel, R. L., McNeilage, L. J., Surh, C. D., de Water, J. V., Spithill, T. W., Whittingham, S., & Gershwin, M. E. (1988) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.85.19.7317 · Cited by 252 · see citing works
Self-citationKemp, D. J., Coppel, R. L., Cowman, A. F., Saint, R. B., Brown, G. V., & Anders, R. F. (1983) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.80.12.3787 · Cited by 209 · see citing works
Self-citationde Water, J. V., Cooper, A. E., Surh, C. D., Coppel, R. L., Danner, D. J., Ansari, A. A., Dickson, R. E., & Gershwin, M. E. (1989) · New England Journal of Medicine · doi:10.1056/nejm198905253202104 · Cited by 201 · see citing works
Self-citationde Water, J. V., Fregeau, D. R., Davis, P. A., Ansari, A. A., Danner, D. J., Leung, P. S. C., Coppel, R. L., & Gershwin, M. E. (1988) · The Journal of Immunology · doi:10.4049/jimmunol.141.7.2321 · Cited by 187 · see citing works
Self-citationSurh, C. D., Coppel, R. L., & Gershwin, M. E. (1990) · The Journal of Immunology · doi:10.4049/jimmunol.144.9.3367 · Cited by 169 · see citing works
Self-citationSurh, C. D., Roche, T. E., Danner, D. J., Ansari, A. A., Coppel, R. L., Prindiville, T. P., Dickson, E. R., & Gershwin, M. E. (1989) · Hepatology · doi:10.1002/hep.1840100202 · Cited by 123 · see citing works
Self-citationLeung, P. S. C., Chuang, D., Wynn, M. R., Cha, S.-H., Danner, D. J., Ansari, A. A., Coppel, R. L., & Gershwin, E. M. (1995) · Hepatology · doi:10.1002/hep.1840220220 · Cited by 118 · see citing works
Self-citationFregeau, D. R., Roche, T. E., Davis, P. A., Coppel, R. L., & Gershwin, M. E. (1990) · The Journal of Immunology · doi:10.4049/jimmunol.144.5.1671 · Cited by 108 · see citing works
Self-citationSurh, C. D., Danner, D. J., Ahmed, A. A., Coppel, R. L., Mackay, I. R., Dickson, E. R., & Gershwin, M. E. (1989) · Hepatology · doi:10.1002/hep.1840090110 · Cited by 108 · see citing works
Self-citationFregeau, D. R., Davis, P. A., Danner, D. J., Ansari, A. A., Coppel, R. L., Dickson, E. R., & Gershwin, M. E. (1989) · The Journal of Immunology · doi:10.4049/jimmunol.142.11.3815 · Cited by 83 · see citing works
Self-citationFregeau, D. R., Prindiville, T. P., Coppel, R. L., Kaplan, M., Dickson, R. E., & Gershwin, E. M. (1990) · Hepatology · doi:10.1002/hep.1840110611 · Cited by 67 · see citing works
Self-citationDavis, P. A., Leung, P. S. C., Manns, M. P., Kaplan, M., Muñoz, S. J., Gorin, F. A., Dickson, R. E., Krawitt, E. L., Coppel, R. L., & Gershwin, E. M. (1992) · Hepatology · doi:10.1002/hep.1840160505 · Cited by 67 · see citing works
Self-citationSurh, C. D., Cooper, A. E., Coppel, R. L., Leung, P. S., Ahmed, A. A., Dickson, R. E., & Gershwin, E. M. (1988) · Hepatology · doi:10.1002/hep.1840080217 · Cited by 67 · see citing works
Self-citationHummel, K. B., Litwer, S., Bradford, A. P., Aitken, A., Danner, D. J., & Yeaman, S. J. (1988) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)68766-6 · Cited by 56 · see citing works
Site-directed mutagenesis of lysine within the immunodominant autoepitope of PDC-E2
Leung, P. S. C., Iwayama, T., Coppel, R. L., & Gershwin, E. M. (1990) · Hepatology · doi:10.1002/hep.1840120612 · Cited by 49 · see citing works
Self-citationCharacterization of cDNAs encoding human pyruvate dehydrogenase alpha subunit.
Ho, L., Wexler, I. D., Liu, T. C., Thekkumkara, T., & Patel, M. S. (1989) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.86.14.5330 · Cited by 49 · see citing works
Nakano, K., Matuda, S., Yamanaka, T., Tsubouchi, H., Nakagawa, S., Titani, K., Ohta, S., & Miyata, T. (1991) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(18)55164-4 · Cited by 40 · see citing works
Self-citationMolecular cloning of the liver-specific rat F antigen.
Gershwin, M. E., Coppel, R. L., Bearer, E. A., PETERSON, M. G., Sturgess, A. D., & Mackay, I. R. (1987) · The Journal of Immunology · doi:10.4049/jimmunol.139.11.3828 · Cited by 39 · see citing works
Self-citationWilliamson, J. M., Chalmers, D. M., Clayden, A. D., Dixon, M. F., Ruddell, W. S., & Losowsky, M. S. (1985) · Journal of Clinical Pathology · doi:10.1136/jcp.38.9.1007 · Cited by 36 · see citing works
Mm, K. (1987) · PubMed · Cited by 36 · see citing works
Predominance of IgG3 subclass in primary cirrhosis.
Riggione, O., Stokes, R. P., & Thompson, R. (1983) · BMJ · doi:10.1136/bmj.286.6370.1015-a · Cited by 25 · see citing works
Specific reactivity of recombinant human PDC-E1α in primary biliary cirrhosis
Iwayama, T., Leung, P., Coppel, R. L., Roche, T. E., Patel, M. S., Mizushima, Y., Nakagawa, T., Dickson, R. E., & Gershwin, M. E. (1991) · Journal of Autoimmunity · doi:10.1016/0896-8411(91)90172-9 · Cited by 24 · see citing works
Self-citationNakano, K., Matuda, S., Sakamoto, T., Takase, C., Nakagawa, S., Ohta, S., Ariyama, T., Inazawa, J., Abe, T., & Miyata, T. (1993) · Biochimica et Biophysica Acta (BBA) - Gene Structure and Expression · doi:10.1016/0167-4781(93)90002-u · Cited by 23 · see citing works
Self-citationLitwer, S., & Danner, D. J. (1985) · Biochemical and Biophysical Research Communications · doi:10.1016/0006-291x(85)91333-6 · Cited by 20 · see citing works
Paraproteins in Primary Biliary Cirrhosis
Hendrick, A. M., Mitchison, H. C., Bird, A. G., & James, O. (1986) · QJM · doi:10.1093/oxfordjournals.qjmed.a068025 · Cited by 13 · see citing works
Molecular cloning of the liver-specific rat F antigen
Gershwin, M., Coppel, R. L., Bearer, E. A., Peterson, M. G., Sturgess, A. D., & Ir, M. (1988) · The Journal of Immunology · doi:10.4049/jimmunol.140.7.2477.b · Cited by 5 · see citing works
Self-citationCloning and identification of autoantigens
Leung, P. S. C., & Gershwin, M. E. (1992) · ImmunoMethods · doi:10.1016/s1058-6687(05)80011-7 · Cited by 3 · see citing works
Self-citation
Flagged first, then by citations
Research works printed but not linked: 9
- Yeaman SJ, Fussey SPM, Danner DJ, James OFW, Mutimer 7. Fregeau DR, Davis PA, Danner DJ, Ansari A, Coppel RL, Dick- human PDC-E1a in primary biliary cirrhosis. J Autoimmunity 1991;4:769-778.
- Fregeau DR, Roche TE, Davis PA, Coppel R, Gershwin ME. Inhi- bition of pyruvate dehydrogenase complex activity by autoanti- HEPATOLOGY 1990;11:975-981.
- Nakano K, Matuda S, Yamanaka T, Tsubouchi H, Nakagawa S, bodies specific for E1a, a non-lipoic acid containing mitochon- drial enzyme. J Immunol 1990;144:1671-1676.
- Titani K, Ohta S, et al. Purification and molecular cloning of succinyltransferase of the rat a-ketoglutarate dehydrogenase 9. Surh CD, Roche TE, Danner DJ, Ansari A, Coppel RL, Prindiville T, Dickson ER, et al. Antimitochondrial autoantibodies in pri- complex. J Biol Chem 1991;266:19013-19017.
- Gershwin ME, Coppel RL, Bearer E, Peterson MG, Sturgess A, drolipoamide acetyltransferase. J Exp Med 1988;167:1791-1799.
- tein structure. J Biol Chem 1988;263:6165-6168.
- Surh CD, Danner DJ, Aftab A, Coppel RL, Mackay IR, Dickson plex family. TIBS 1986;11:293-296.
- Iwayama T, Leung PSC, Coppel RL, Roche TE, Patel MS, Mizu- ase: cDNA cloning and localization on chromosome 14q24.2- q24.3. Biochim Biophys Acta 1993;1216:360-368.
- hepa WBS: Hepatology 15273350, 1996, 3, Downloaded from https://aasldpubs.onlinelibrary.wiley.com/doi/10.1002/hep.510230307, Wiley Online Library on [14/08/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Cited by
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Lindor, K. D., Gershwin, E. M., Poupon, R., Kaplan, M., Bergasa, N. V., & Heathcote, J. E. (2009) · Hepatology · doi:10.1002/hep.22906 · Cited by 1,099 · see citing works
Lindor, K. D., Bowlus, C. L., Boyer, J., Levy, C., & Mayo, M. (2019) · Hepatology · doi:10.1002/hep.30145 · Cited by 913 · see citing works
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Hirschfield, G. M., & Gershwin, M. E. (2013) · Annual Review of Pathology: Mechanisms of Disease · doi:10.1146/annurev-pathol-020712-164014 · Cited by 315 · see citing works
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Gershwin, E. M., & Mackay, I. R. (2007) · Hepatology · doi:10.1002/hep.22042 · Cited by 273 · see citing works
Lleo, A., Wang, G.-Q., Gershwin, M. E., & Hirschfield, G. M. (2020) · The Lancet · doi:10.1016/s0140-6736(20)31607-x · Cited by 253 · see citing works
Self-citationShimoda, S., de Water, J. V., Ansari, A. A., Nakamura, M., Ishibashi, H., Coppel, R. L., Lake, J. R., Keeffe, E. B., Roche, T. E., & Gershwin, M. E. (1998) · Journal of Clinical Investigation · doi:10.1172/jci4213 · Cited by 250 · see citing works
Self-citationOdin, J. A., Huebert, R. C., Casciola-Rosen, L., LaRusso, N. F., & Rosen, A. (2001) · Journal of Clinical Investigation · doi:10.1172/jci200110716 · Cited by 220 · see citing works
Miyakawa, H., Tanaka, A., Kikuchi, K., Matsushita, M., Kitazawa, E., Kawaguchi, N., Fujikawa, H., & Gershwin, E. M. (2001) · Hepatology · doi:10.1053/jhep.2001.26514 · Cited by 200 · see citing works
Hohenester, S. D., Elferink, R. P. J. O., & Beuers, U. H. (2009) · Seminars in Immunopathology · doi:10.1007/s00281-009-0164-5 · Cited by 176 · see citing works
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Mattalia, A., Quaranta, S., Leung, P. S. C., Bauducci, M., Van de Water, J., Calvo, P. L., Danielle, F., Rizzetto, M., Ansari, A., Coppel, R. L., et al. (1998) · Hepatology · doi:10.1002/hep.510270303 · Cited by 146 · see citing works
Self-citationMa, W.-T., Chang, C. C.-C., Gershwin, M. E., & Lian, Z. (2017) · Journal of Autoimmunity · doi:10.1016/j.jaut.2017.07.003 · Cited by 145 · see citing works
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Self-citationLeung, P. S. C., Quan, C., Park, O., de Water, J. V., Kurth, M. J., Nantz, M. H., Ansari, A. A., Coppel, R. L., Lam, K. S., & Gershwin, M. E. (2003) · The Journal of Immunology · doi:10.4049/jimmunol.170.10.5326 · Cited by 124 · see citing works
Self-citationMyers, R. P., Swain, M. G., Lee, S. S., Shaheen, A. A., & Burak, K. W. (2013) · The American Journal of Gastroenterology · doi:10.1038/ajg.2013.51 · Cited by 123 · see citing works
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Yoshida, K., Yang, G., Zhang, W., Tsuda, M., Tsuneyama, K., Moritoki, Y., Ansari, A. A., Okazaki, K., Lian, Z., Coppel, R. L., et al. (2009) · Hepatology · doi:10.1002/hep.23132 · Cited by 117 · see citing works
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Self-citationAntimitochondrial and other autoantibodies
Bogdanos, D. P., Baum, H., & Vergani, D. (2003) · Clinics in Liver Disease · doi:10.1016/s1089-3261(03)00104-1 · Cited by 99 · see citing works
Shimoda, S., Miyakawa, H., Nakamura, M., Ishibashi, H., Kikuchi, K., Kita, H., Niiro, H., Arinobu, Y., Ono, N., Mackay, I. R., et al. (2008) · Journal of Autoimmunity · doi:10.1016/j.jaut.2008.05.003 · Cited by 92 · see citing works
Self-citationFamilial Primary Biliary Cirrhosis in Hiroshima
Tsuji, K., Watanabe, Y., de Water, J. V., Nakanishi, T., Kajiyama, G., Parikh‐Patel, A., Coppel, R. L., & Gershwin, M. E. (1999) · Journal of Autoimmunity · doi:10.1006/jaut.1999.0299 · Cited by 87 · see citing works
Self-citationPathogen infections and primary biliary cholangitis
Tanaka, A., Leung, P. S. C., & Gershwin, M. E. (2018) · Clinical & Experimental Immunology · doi:10.1111/cei.13198 · Cited by 87 · see citing works
Self-citationMigliaccio, C. T., Nishio, A., de Water, J. V., Ansari, A. A., Leung, P. S. C., Nakanuma, Y., Coppel, R. L., & Gershwin, M. E. (1998) · The Journal of Immunology · doi:10.4049/jimmunol.161.10.5157 · Cited by 83 · see citing works
Self-citationReynoso-Paz, S., Leung, P. S. C., de Water, J. V., Tanaka, A., Munoz, S., Bass, N. M., Lindor, K. D., Donald, P. J., Coppel, R. L., Ansari, A. A., et al. (2000) · Hepatology · doi:10.1002/hep.510310106 · Cited by 81 · see citing works
Self-citationWakabayashi, K., Yoshida, K., Leung, P. S. C., Moritoki, Y., Yang, G.-X., Tsuneyama, K., Lian, Z.-X., Hibi∥, T., Ansari, A. A., Wicker, L. S., et al. (2008) · Clinical & Experimental Immunology · doi:10.1111/j.1365-2249.2008.03837.x · Cited by 78 · see citing works
Self-citationDubel, L., Tanaka, A., Leung, P. S. C., de Water, J. V., Coppel, R. L., Roche, T. E., Johanet, C., Motokawa, Y., Ansari, A. A., & Gershwin, E. M. (1999) · Hepatology · doi:10.1002/hep.510290403 · Cited by 78 · see citing works
Self-citationNishio, A., de Water, J. V., Leung, P. S. C., Joplin, R., Neuberger, J., Lake, J. R., Björkland, A., Tötterman, T. H., Peters, M. G., Worman, H. J., et al. (1997) · Hepatology · doi:10.1002/hep.510250506 · Cited by 77 · see citing works
Self-citationImmunological abnormalities in patients with primary biliary cholangitis.
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Bellucci, R., Wu, C. J., Chiaretti, S., Weller, E. A., Davies, F. E., Alyea, E. P., Dranoff, G., Anderson, K. C., Munshi, N. C., & Ritz, J. (2003) · Blood · doi:10.1182/blood-2003-07-2559 · Cited by 75 · see citing works
Wang, J. J., Yang, G.-X., Zhang, W. C., Lu, L., Tsuneyama, K., Kronenberg, M., Véla, J. L., Lopez-Hoyos, M., He, X.-S., Ridgway, W. M., et al. (2014) · Clinical and Experimental Immunology · doi:10.1111/cei.12224 · Cited by 74 · see citing works
Self-citationShigematsu, H., Shimoda, S., Nakamura, M., Matsushita, S., Nishimura, Y., Sakamoto, N., Ichiki, Y., Niho, Y., Gershwin, E. M., & Ishibashi, H. (2000) · Hepatology · doi:10.1053/jhep.2000.18714 · Cited by 72 · see citing works
Autoimmune cholangitis and primary biliary cirrhosis ? an autoimmune enigma
Kinoshita, H., Omagari, K., Whittingham, S., Kato, Y., Ishibashi, H., Sugi, K., Yano, M., Kohno, S., Nakanuma, Y., Penner, E., et al. (1999) · Liver International · doi:10.1111/j.1478-3231.1999.tb00021.x · Cited by 69 · see citing works
Self-citationEnvironment and primary biliary cirrhosis: Electrophilic drugs and the induction of AMA
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Self-citationRigopoulou, E., Davies, E., Bogdanos, D.-P., Liaskos, C., Mytilinaiou, M., Koukoulis, G., Dalekos, G., & Vergani, D. (2007) · Liver international (Print) · doi:10.1111/j.1478-3231.2007.01586.x · Cited by 67 · see citing works
Rong, G., Zhong, R., Lleò, A., Leung, P. S. C., Bowlus, C. L., Yang, G., Yang, C., Coppel, R. L., Ansari, A. A., Cuebas, D. A., et al. (2011) · Hepatology · doi:10.1002/hep.24355 · Cited by 63 · see citing works
Self-citationPathogenesis of primary biliary cirrhosis
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Primary biliary cirrhosis: What do autoantibodies tell us?
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Pathogenesis of Primary Biliary Cirrhosis
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Self-citationAutoantigens in primary biliary cirrhosis
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Gabeta, S., Norman, G., Liaskos, C., Papamichalis, P., Zografos, T., Garagounis, A., Rigopoulou, E., & Dalekos, G. (2007) · Journal of Clinical Immunology · doi:10.1007/s10875-007-9092-0 · Cited by 62 · see citing works
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Self-citationEnvironmental basis of primary biliary cholangitis
Tanaka, A., Leung, P. S. C., & Gershwin, M. E. (2018) · Experimental Biology and Medicine · doi:10.1177/1535370217748893 · Cited by 51 · see citing works
Self-citationKitahata, S., Yamamoto, Y., Yoshida, O., Tokumoto, Y., Kawamura, T., Furukawa, S., Kumagi, T., Hirooka, M., Takeshita, E., Abe, M., et al. (2021) · Scientific Reports · doi:10.1038/s41598-021-99314-9 · Cited by 47 · see citing works
Kawata, K., Yang, G., Ando, Y., Tanaka, H., Zhang, W., Kobayashi, Y., Tsuneyama, K., Leung, P. S. C., Lian, Z., Ridgway, W. M., et al. (2013) · Hepatology · doi:10.1002/hep.26418 · Cited by 46 · see citing works
Self-citationEvolution of our understanding of PBC
Tanaka, A., Leung, P. S. C., & Gershwin, M. E. (2018) · Best Practice & Research Clinical Gastroenterology · doi:10.1016/j.bpg.2018.05.008 · Cited by 40 · see citing works
Self-citationLeung, P. S. C., Choi, J., Yang, G., Woo, E., Kenny, T. P., & Gershwin, M. E. (2016) · Expert Review of Molecular Diagnostics · doi:10.1586/14737159.2016.1164038 · Cited by 39 · see citing works
Self-citationClinical Management of Primary Biliary Cholangitis—Strategies and Evolving Trends
Gao, L., Wang, L., Woo, E., He, X., Yang, G., Bowlus, C., Leung, P. S. C., & Gershwin, M. E. (2020) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-019-08772-7 · Cited by 39 · see citing works
Self-citationKawata, K., Kobayashi, Y., Gershwin, M. E., & Bowlus, C. L. (2012) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-012-8324-0 · Cited by 38 · see citing works
Self-citationAnimal Models of Primary Biliary Cirrhosis
Chuang, Y., Ridgway, W. M., Ueno, Y., & Gershwin, M. E. (2008) · Clinics in Liver Disease · doi:10.1016/j.cld.2008.02.011 · Cited by 37 · see citing works
Self-citationToward solving the etiological mystery of primary biliary cholangitis
Tanaka, A., Leung, P. S. C., Young, H. A., & Gershwin, M. E. (2017) · Hepatology Communications · doi:10.1002/hep4.1044 · Cited by 37 · see citing works
Self-citationWang, J., Budamagunta, M. S., Voss, J. C., Kurth, M. J., Lam, K. S., Lü, L., Kenny, T. P., Bowlus, C. L., Kikuchi, K., Coppel, R. L., et al. (2013) · The Journal of Immunology · doi:10.4049/jimmunol.1301092 · Cited by 36 · see citing works
Self-citationTanaka, A., Nezu, S., Uegaki, S., Mikami, M., Okuyama, S., KAWAMURA, N., Aiso, M., Gershwin, M. E., Takahashi, S., Selmi, C. F., et al. (2007) · Annals of the New York Academy of Sciences · doi:10.1196/annals.1381.028 · Cited by 35 · see citing works
Self-citationTanaka, T., Zhang, W., Sun, Y. H., Shuai, Z., Chida, A. S., Kenny, T. P., Yang, G., Sanz, I., Ansari, A. A., Bowlus, C. L., et al. (2017) · Hepatology · doi:10.1002/hep.29245 · Cited by 34 · see citing works
Self-citationImmunopathogenesis of Gastrointestinal and Hepatobiliary Diseases
Galperin, C. (1997) · JAMA · doi:10.1001/jama.1997.03550220152020 · Cited by 30 · see citing works
Self-citationMattalia, A., Lüttig, B., Rosina, F., Leung, P. S. C., de Water, J. V., Bauducci, M., Ciancio, A., Böker, K. H. W., Worman, H. J., Cooper, R. L., et al. (1997) · Journal of Autoimmunity · doi:10.1006/jaut.1997.0156 · Cited by 31 · see citing works
Self-citationAnimal Models of Primary Biliary Cirrhosis: Materials and Methods
Leung, P. S. C., Yang, G. X., Dhirapong, A., Tsuneyama, K., Ridgway, W. M., & Gershwin, M. E. (2012) · Methods in molecular biology · doi:10.1007/978-1-60761-720-4_14 · Cited by 30 · see citing works
Self-citationModulation of 2-Oxoglutarate Dehydrogenase Complex by Inorganic Phosphate, Mg2+, and Other Effectors
Rodríguez‐Zavala, J. S., Pardo, J. P., & Moreno‐Sánchez, R. (2000) · Archives of Biochemistry and Biophysics · doi:10.1006/abbi.2000.1856 · Cited by 30 · see citing works
Autoimmune tests in primary biliary cirrhosis
Strassburg, C. P., & Manns, M. P. (2000) · Best Practice & Research Clinical Gastroenterology · doi:10.1053/bega.2000.0105 · Cited by 29 · see citing works
Molecular diagnostics of primary biliary cirrhosis
Rigopoulou, E. I., & Dalekos, G. N. (2008) · Expert Opinion on Medical Diagnostics · doi:10.1517/17530059.2.6.621 · Cited by 28 · see citing works
Koike, K., Ishibashi, H., & Koike, M. (1998) · Hepatology · doi:10.1002/hep.510270602 · Cited by 27 · see citing works
Pathogenesis of primary biliary cirrhosis
Kita, H., Nalbandian, G., Keeffe, E. B., Coppel, R. L., & Gershwin, M. E. (2003) · Clinics in Liver Disease · doi:10.1016/s1089-3261(03)00096-5 · Cited by 27 · see citing works
Self-citationShimoda, S., Tsuneyama, K., Kikuchi, K., Harada, K., Nakanuma, Y., Nakamura, M., Ishibashi, H., Hisamoto, S., Niiro, H., Leung, P. S. C., et al. (2012) · Clinical & Experimental Immunology · doi:10.1111/j.1365-2249.2012.04581.x · Cited by 26 · see citing works
Self-citationAnti-mitochondrial autoantibodies
Fritzler, M. J., & Manns, M. P. (2002) · Clinical and Applied Immunology Reviews · doi:10.1016/s1529-1049(02)00058-2 · Cited by 26 · see citing works
Is There A Serological Difference Between Men and Women With Primary Biliary Cirrhosis?
Nalbandian, G., de Water, J. V., Gish, R. G., Manns, M. P., Coppel, R. L., Rudich, S. M., Prindiville, T. P., & Gershwin, E. M. (1999) · The American Journal of Gastroenterology · doi:10.1111/j.1572-0241.1999.01380.x · Cited by 24 · see citing works
Self-citationMoritoki, Y., Tsuda, M., Tsuneyama, K., Zhang, W., Yoshida, K., Lian, Z.-X., Yang, G., Ridgway, W. M., Wicker, L. S., Ansari, A. A., et al. (2011) · Cellular Immunology · doi:10.1016/j.cellimm.2011.01.005 · Cited by 23 · see citing works
Self-citationAnti-mitochondrial M2 antibody-positive autoimmune hepatitis
TOMIZAWA, M., SHINOZAKI, F., FUGO, K., MOTOYOSHI, Y., SUGIYAMA, T., YAMAMOTO, S., KISHIMOTO, T., & ISHIGE, N. (2015) · Experimental and Therapeutic Medicine · doi:10.3892/etm.2015.2694 · Cited by 22 · see citing works
MacDonald, M. J., Husain, R. D., Hoffmann-Benning, S., & Baker, T. R. (2004) · Journal of Biological Chemistry · doi:10.1074/jbc.m314148200 · Cited by 22 · see citing works
Xenobiotics and loss of tolerance in primary biliary cholangitis
Wang, J. (2015) · World Journal of Gastroenterology · doi:10.3748/wjg.v22.i1.338 · Cited by 21 · see citing works
Harada, K., Sudo, Y., Kono, N., Ozaki, S., Tsuneyama, K., Gershwin, E. M., & Nakanuma, Y. (1999) · Hepatology · doi:10.1002/hep.510300145 · Cited by 21 · see citing works
Mitochondrial Antigens as Targets of Cellular and Humoral Auto-Immunity in Primary Biliary Cirrhosis
Ichiki, Y., Selmi, C. F., Shimoda, S., Ishibashi, H., Gordon, S. C., & Gershwin, M. E. (2005) · Clinical Reviews in Allergy & Immunology · doi:10.1385/criai:28:2:083 · Cited by 19 · see citing works
Self-citationImmunopathogenesis of cholestatic autoimmune liver diseases
Medina, J., Jones, E. A., García‐Monzón, C., & Moreno‐Otero, R. (2001) · European Journal of Clinical Investigation · doi:10.1046/j.1365-2362.2001.00765.x · Cited by 19 · see citing works
Pathological Features of New Animal Models for Primary Biliary Cirrhosis
Tsuneyama, K., Moritoki, Y., Kikuchi, K., & Nakanuma, Y. (2011) · International Journal of Hepatology · doi:10.1155/2012/403954 · Cited by 18 · see citing works
Berdichevski, T., Cohen‐Ezra, O., Pappo, O., & Ben‐Ari, Z. (2016) · Hepatology Research · doi:10.1111/hepr.12809 · Cited by 18 · see citing works
The pyruvate dehydrogenase complex as a target autoantigen in primary biliary cirrhosis.
Nishio, A., Coppel, R., Ishibashi, H., & Gershwin, M. (2000) · Baillière's Best Practice & Research : Clinical Gastroenterology · doi:10.1053/bega.2000.0102 · Cited by 17 · see citing works
Self-citationPrimary Biliary Cholangitis in Males
Shaker, M., Mansour, N., & John, B. V. (2022) · Clinics in Liver Disease · doi:10.1016/j.cld.2022.06.008 · Cited by 18 · see citing works
High sensitivity of a novel ELISA for anti-M2 in primary biliary cirrhosis
Miyakawa, H., Kikuchi, K., Kang, J., Kawaguchi, N., Yajima, R., Ito, Y., & Maekubo, H. (2001) · Journal of Gastroenterology · doi:10.1007/s005350170151 · Cited by 17 · see citing works
5 Immunopathology of primary biliary cirrhosis
Galperin, C., & Gershwin, M. E. (1996) · Baillière s Clinical Gastroenterology · doi:10.1016/s0950-3528(96)90053-6 · Cited by 17 · see citing works
Self-citationAutoimmune liver disease and multiple sclerosis: state of the art and future perspectives
Villani, R., Serviddio, G., Avolio, C., Cassano, T., & D’Amico, E. (2023) · Clinical and Experimental Medicine · doi:10.1007/s10238-023-01128-8 · Cited by 16 · see citing works
Cutting-Edge Issues in Primary Biliary Cirrhosis
Folci, M., Meda, F., Gershwin, M. E., & Selmi, C. F. (2011) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-011-8253-3 · Cited by 16 · see citing works
Self-citationXenobiotics and autoimmunity: does acetaminophen cause primary biliary cirrhosis?
Leung, P. S. C., Lam, K. S., Kurth, M. J., Coppel, R. L., & Gershwin, M. E. (2012) · Trends in Molecular Medicine · doi:10.1016/j.molmed.2012.07.005 · Cited by 15 · see citing works
Self-citationB cell depletion in treating primary biliary cirrhosis: Pros and cons
Yin, Y. (2012) · World Journal of Gastroenterology · doi:10.3748/wjg.v18.i30.3938 · Cited by 15 · see citing works
Prevalence of Celiac Disease in Patients with Primary Biliary Cholangitis
Callichurn, K., Cvetkovic, L., Therrien, A., Vincent, C., Hétu, P., & Bouin, M. (2019) · Journal of the Canadian Association of Gastroenterology · doi:10.1093/jcag/gwz039 · Cited by 15 · see citing works
Primary biliary cirrhosis and liver transplantation
Akamatsu (2012) · Intractable & Rare Diseases Research · doi:10.5582/irdr.2012.v1.2.66 · Cited by 15 · see citing works
Mitochondrion: A Promising Target for Nanoparticle-Based Vaccine Delivery Systems
Wen, R. M., Umeano, A. C., Francis, L., Sharma, N. D., Tundup, S., & Dhar, S. (2016) · Vaccines · doi:10.3390/vaccines4020018 · Cited by 14 · see citing works
Miyakawa, H. (1999) · Hepatology Research · doi:10.1016/s1386-6346(98)00088-6 · Cited by 14 · see citing works
An Update on the Treatment and Follow-up of Patients with Primary Biliary Cholangitis
Burman, B. E., Jhaveri, M. A., & Kowdley, K. V. (2017) · Clinics in Liver Disease · doi:10.1016/j.cld.2017.06.005 · Cited by 13 · see citing works
Autoimmune Markers in Primary Biliary Cholangitis
Shah, S. K., & Bowlus, C. L. (2023) · Clinics in Liver Disease · doi:10.1016/j.cld.2023.07.002 · Cited by 12 · see citing works
Mawardi, M., Alalwan, A. M., Fallatah, H. I., Abaalkhail, F. A., Hasosah, M. Y., Shagrani, M. A., Alghamdi, M., & Alghamdi, A. (2021) · Saudi Journal of Gastroenterology · doi:10.4103/sjg.sjg_112_21 · Cited by 12 · see citing works
New insights on the role of human leukocyte antigen complex in primary biliary cholangitis
Mulinacci, G., Palermo, A., Gerussi, A., Asselta, R., Gershwin, M. E., & Invernizzi, P. (2022) · Frontiers in Immunology · doi:10.3389/fimmu.2022.975115 · Cited by 12 · see citing works
Self-citationDecreased serum DNase1-activity in patients with autoimmune liver diseases
Gatselis, N., Vakrakou, A., Zachou, K., Androutsakos, T., Azariadis, K., Hatzis, G., Manoussakis, M., & Dalekos, G. (2017) · Autoimmunity · doi:10.1080/08916934.2017.1279610 · Cited by 11 · see citing works
Miyakawa, H., Abe, K., Kitazawa, E., Kikuchi, K., Fujikawa, H., Matsushita, M., Kawaguchi, N., & Kako, M. (1999) · Autoimmunity · doi:10.3109/08916939908994755 · Cited by 12 · see citing works
Lazaridis, K. N., & Lindor, K. D. (1999) · Current Treatment Options in Gastroenterology · doi:10.1007/s11938-999-0051-0 · Cited by 12 · see citing works
Miyakawa, H. (2000) · Hepatology Research · doi:10.1016/s1386-6346(99)00079-0 · Cited by 11 · see citing works
Miyakawa, H. (1998) · Hepatology Research · doi:10.1016/s1386-6346(98)00054-0 · Cited by 10 · see citing works
An Integrated Pathogenetic Model of Primary Biliary Cholangitis
Kouroumalis, E., Tsomidis, I., & Voumvouraki, A. (2025) · Livers · doi:10.3390/livers5020015 · Cited by 7 · see citing works
Mitochondria and autoimmunity in primary biliary cirrhosis
Ichiki, Y., Leung, P. S. C., Ishibashi, H., Coppel, R. L., Ansari, A. A., & Gershwin, M. E. (2004) · Mitochondrion · doi:10.1016/j.mito.2004.07.024 · Cited by 6 · see citing works
Self-citationBone marrow findings in patients with autoimmune liver diseases
Tsikrikoni, A., Rigopoulou, E. I., Zachou, K., Liaskos, C., Kyriakou, D., & Dalekos, G. N. (2008) · Journal of Gastroenterology and Hepatology · doi:10.1111/j.1440-1746.2008.05309.x · Cited by 6 · see citing works
A case of latent primary biliary cirrhosis
Tsuji, K. (2004) · Hepatology Research · doi:10.1016/j.hepres.2003.09.006 · Cited by 6 · see citing works
Shibata, M., Morizane, T., Tanaka, A., Onozuka, Y., Uchida, T., Gershwin, M. E., & Mitamura, K. (2001) · Journal of Gastroenterology and Hepatology · doi:10.1046/j.1440-1746.2001.02413.x · Cited by 6 · see citing works
Self-citation
- Authors
- Moteki, S., Leung, P. S., Dickson, E. R., Van Thiel, D. H., Galperin, C., Buch, T., Alarcon-Segovia, D., Kershenobich, D., Kawano, K., Coppel, R. L., Matuda, S., & Gershwin, M. E.
- Affiliations
- University of California, Davis (US)
- Venue
- Hepatology
- Published
- 1996-03
- Volume
- 23
- Issue
- 3
- Pages
- 436-444
- ISSN
- 0270-9139 (ISSN-L)
- OpenAlex
- W2143495513
- Cited by
- 133
- References
- 36
- Citations by year
- 2025: 1 · 2024: 3 · 2023: 2 · 2022: 3 · 2021: 3 · 2020: 2 · 2019: 5 · 2018: 4 · 2017: 7 · 2016: 4 · 2015: 3 · 2014: 1 · 2013: 6 · 2012: 7
- FWCI
- 5.20
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Hepatology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (5r29ai031585-05)
- National Institute of Allergy and Infectious Diseases (AI 31585)
- National Institute of Diabetes and Digestive and Kidney Diseases (DK39558)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Rheumatology › Systemic Lupus Erythematosus Research
- Health Sciences › Medicine › Pathology and Forensic Medicine › Autoimmune Bullous Skin Diseases
- Keywords
- primary biliary cirrhosis, autoantibodies, 2-oxoglutarate dehydrogenase, E2 subunit, epitope mapping, mitochondrial autoantigens, antimitochondrial antibodies, lipoyl domain, conformational epitopes, immunodominant epitopes, pyruvate dehydrogenase complex, recombinant fusion protein, isotype distribution, autoantigen specificity
- MeSH terms
- Animals, Antigen-Antibody Reactions, Epitopes, Autoantibodies, Autoantigens, Base Sequence, Humans, Ketoglutarate Dehydrogenase Complex, Liver Cirrhosis, Biliary, Mitochondria, Molecular Sequence Data, Recombinant Fusion Proteins, Immunoblotting, Rats
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