The immunopathology of primary biliary cirrhosis: Thoughts for the millennium

Archivum Immunologiae et Therapiae Experimentalis

Volumn 48, Issue 1, 2000, Pages 1-10

  Sasaki, Motoko ^a,b   Ansari, Aftab A ^c   Nakanuma, Yasuni ^b   Coppel, Ross L ^e   Keeffe, Emmet B ^d   Gershwin, M Eric ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b KANAZAWA UNIVERSITY   (Japan)

^c EMORY UNIVERSITY SCHOOL OF MEDICINE   (United States)

^d STANFORD UNIVERSITY SCHOOL OF MEDICINE   (United States)

^e NONE

Author keywords

Autoimmunity; Mitochondria; Primary biliary cirrhosis; Pyruvate dehydrogenase

Indexed keywords

AUTOANTIBODY; AUTOANTIGEN; CELL ADHESION MOLECULE; CYTOKINE; HLA ANTIGEN; LYMPHOCYTE ANTIGEN RECEPTOR;

APOPTOSIS; AUTOIMMUNITY; BILIARY CIRRHOSIS; EPITHELIUM; FEMALE; GENETICS; HUMAN; IMMUNOLOGY; INFLAMMATION; MALE; METABOLISM; MITOCHONDRION; PATHOLOGY; REVIEW; T LYMPHOCYTE;

APOPTOSIS; AUTOANTIBODIES; AUTOANTIGENS; AUTOIMMUNITY; CELL ADHESION MOLECULES; CYTOKINES; EPITHELIUM; FEMALE; HLA ANTIGENS; HUMANS; INFLAMMATION; LIVER CIRRHOSIS, BILIARY; MALE; MITOCHONDRIA; RECEPTORS, ANTIGEN, T-CELL, ALPHA-BETA; T-LYMPHOCYTES;

EID: 0033630004     PISSN: 0004069X     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (81)

1. ADAMS D., HUBSCHER S., SHAW J., JOHNSON G., BABBS C., ROTHLEIN R and NEUBERGER J. (1991): Increased expression of intercellular adhesion molecule-1 (ICAM-1) on bile ducts in primary biliary cirrhosis and primary sclerosing cholangitis. Hepatology, 14, 426-431.
2. AYRES R., NEUBERGER J., SHAW J., JOPLIN R. and ADAMS D. (1993): Intercellular adhesion molecule-1 and MHC antigens on human intrahepatic bile duct cells: effect of pro-inflammatory cytokines. Gut, 34, 1245-1249.
3. BALLARDINI G., MIRAKIAN R., BIANCHI F., PISI E., DONIACH D. and BOTTAZZO G. (1984): Abberant expression of HLA-DR antigens on bile duct epithelium in primary biliary cirrhosis. Lancet, 2, 1009-1013.
4. BANDIN O., COUVALIN J., POUPON R., DUBEL L., HOMBERG J. and JOHANNET C. (1996): Specificity and sensitivity of gp210 autoantibodies detected using an enzyme-linked immunosorbent assay and a synthetic polypeptide in the diagnosis of primary biliary cirrhosis. Hepatology, 23, 1020-1024.
5. BASSENDINE M., FUSSEY S., MUTIMER D., JAMES O. and YEAMAN S. (1989): Identification and classification of four M2 mitochondrial autoantigens in primary biliary cirrhosis. Semin. Liver Dis., 9, 124-131.
6. CHA S., LEUNG P., COPPEL R., VAN DE WATER J., ANSARI A. and GERSHWIN M. (1994): Heterogeneity of combinatorial human autoantibodies against PDC-E2 and biliary epithelial cells in paients with primary biliary cirrhosis. Hepatology, 20, 574-583.
7. CHARLTON B. and LAFFERTY K. (1995): The Th1/Th2 balance in autoimmunity. Curr. Opin. Immunol., 7, 793-798.
8. COLUCCI G., SCHAFFNER F. and PARONETTO F. (1986): In situ characterization of the cell-surface antigens of the mononuclear cell infiltrate and bile duct epithelium in primary biliary cirrhosis. Clin. Immunol. Immunopathol., 41, 741-747.
9. COPPEL R., MCNEILAGE L., SURH C., VAN DE WATER J., SPITHILL T., WHITTINGHAM S. and GERSHWIN M. (1988): Primary structure of the human M2 motochondrial autoantigen of primary biliary cirrhosis. Proc. Natl. Acad. Sci. USA, 85, 7317-7321.
10. DANPURE C. J. (1998): The molecular basis of alanine: glyoxylate aminotransferase mistargeting: the most common single cause of primary hyperoxaluria type I. J. Nephrol., 11 (suppl. 1), 8-12.
11. DEMETRIS A., SEVER R., KAKIZOE S., OGUMA S., STARZL T. and JAFFE R. (1989): S100 protein positive dendritic cells in primary biliary cirrhosis and other chronic inflammatory liver diseases: relevance to pathogenesis? Am. J. Pathol., 134.
12. DONALDSON P., DOHERTY D., UNDERHILL J. and WILLIAMS R. (1994): The molecular genetics of autoimmune liver disease. Hepatology, 20, 225-239.
13. FLANNERY G., BURROUGHS A., BUTLER P., CHELLIAH J., HAMILTON-MILLER J., BRUMFITT W. and BAUM H. (1989): Antimitochondrial antibodies in primary biliary cirrhosis recognize both specific peptides and shared epitopes of the M2 family of antigens. Hepatology, 10, 370-374.
14. FREGEAU D., ROCHE T., DAVIS P., COPPEL R. and GERSHWIN M. (1990): Primary biliary cirrhosis. Inhibition of pyruvate dehydrogenase complex activity by autoantibodies specific for E1 alpha, a non-lipoic acid containing mitochondrial enzyme. J. Immunol., 144, 1671-1676.
15. FUSSEY S., ALI S., GUEST J., JAMES O., BASSENDINE M. and YEAMAN S. (1990): Reactivity of primary biliary cirrhosis sera with Escherichia coli dihydrolipoamide acetyltransferase (E2p): characterization of the main immunogenic region. Proc. Natl. Acad. Sci. USA, 87, 3987-3991.
16. FUSSEY S., BASSENDINE M. and FITTES D. (1989): The E1 alpha and beta subunits of the pyruvate dehydrogenase complex are M2'd' and M2'e' autoantigens in primary biliary cirrhosis. Clin. Sci., 77, 365-368.
17. FUSSEY S., GUEST J., JAMES O., BASSENDINE M. and YEAMAN S. (1988): Identification and analysis of the major M2 autoantigens in primary biliary cirrhosis. Proc. Natl. Acad. Sci. USA, 85, 8654-8658.
18. GERSHWIN M., MACKAY I., STURGESS A. and COPPEL R. (1987): Identification and specificity of a cDNA encoding the 70 kd mitochondorial antigen recognized in primary biliary cirrhosis. J. Immunol., 138, 3525-3531.
19. GONZALEZ-AMARO R., GARCIS-MONZON C., GARCIA-BUEY L., MORENO-OTERO R., ALONSO J., YAGUE E., PlVEL J., LOPEZ-CABERE M., GERNANDEZ-RUIZ E. and SANCHEZ-MADRID F. (1994): Induction of tumor necrosis factor alpha production by human hepatocytes in chronic viral hepatitis. J. Exp. Med., 179, 841-848.
20. HARADA K., OZAKI S., GERSHWIN M. and NAKANUMA Y. (1997): Enhanced apoptosis related to bile duct loss in primary biliary cirrhosis. Hepatology, 26, 1399-1405.
21. HARADA K., VAN DE WATER J., LEUNG P., COPPEL R., ANSARI A., NAKANUMA Y. and GERSHWIN M. (1997): In situ hybridization of cytokines in primary biliary cirrhosis. Predominance of the Th1 subset. Hepatology, 25, 791-796.
22. HARADA K., VAN DE WATER J., LEUNG P., COPPEL R., NAKANUMA Y. and GERSHWIN M. (1997): In situ nucleic acid hybridization of pyruvate dehydrogenase complex-E2 in primary biliary cirrhosis: pyruvate dehydrogenase complex-E2 messenger RNA is expressed in hepatocytes but not in biliary epithelium. Hepatology, 25, 27-32.
23. HARRIS R., BOWKER-KINLEY M., WU P., JENG J. and POPOV K. (1997): Dihydrolipoamide dehydrogenase complex. DNA-derived amino acid sequence, expression, and reconstitution of the pyruvate dehydrogenase complex. J. Biol. Chem., 272, 6361-6369.
24. HASHIMOTO E., LINDOR K., HOMBURGER H., DICKSON E., CZAJA A., WIESNER R. and LUDWIG J. (1993): Immunohistochemical characterization of hepatic lymphocytes in primary biliary cirrhosis in comparison with primary sclerosing cholangitis and autoimmune chronic active hepatitis. Mayo Clin. Proc., 68, 1049-1055.
25. HIRAISHI K., SUZUKI K., HAKOMORI S. and ADACHI M. (1993): Le (y) antigen expression is correlated with apoptosis (programmed cell death). Glycobiology, 3, 381-390.
26. HOFFMAN R., PAGE G. and SPEMGLER U. (1989): Clonal analysis of liver-derived T cells of patients with primary biliary cirrhosis. Clin. Exp. Immunol., 76, 210-215.
27. ITOH S., ICHIDA T., YOSHIDA T., HAYAKAWA A., UCHIDA M., TASHIRO-ITOH T., MATSUDA Y., ISHIHARA K. and ASAKURA H. (1998): Autoantibodies against a 210 kDa glycoprotein of the nuclear pore complex as a prognostic marker in patients with primary biliary cirrhosis. J. Gastroenterol. Hepatol., 13, 257-265.
28. IWAYAMA T., LEUNG P., COPPEL R., ROCHE T., PATEL M., MIZUSHIMA Y., NAKAGAWA T., DICKSON R. and GERSHWIN M. (1991): Specific reactivity of recombinant human PDC-E1 alpha in primary biliary cirrhosis. J. Autoimm., 4, 769-778.
29. JELNICK D. and LIPSKY P. (1987): Enhancement of human B-cell proliferation and differentiation by tumor necrosis factor alpha and interleukin 1. J. Immunol., 139, 2970-2976.
30. JOPLIN R., BAKES S. and WALLACC L. (1996): Cytotoxicity of lymphocytes for autologous intrahepatic biliary epithelial cells in primary biliary cirrhosis. J. Hepatol., 25, 69.
31. JOPLIN R., LINDSAY J., HUBSCHER S., JOHNSON G., SHAW J., STRAIN A. and NEUBERGER J. (1991): Distribution of dihysrolipoamide acetyltransferase (E2) in the liver and portal lymphnodes of patients with primary biliary cirrhosis: an immunohistochemical study. Hepatology, 14, 442-447.
32. JOPLIN R., WALLACE L. and JOHNSON G. (1994): Distribution of component X of pyruvate dehydrogenase dihydrolipoamide acetyltransferase complex (PDC-X) in liver and salivary gland of patients with primary biliary cirrhosis (PBC) and control. Hepatology, 20, 150A/213.
33. JOPLIN R., WALLACE L., LINDSAY J., PALMER J., YEAMAN S. and NEUBERGER J. (1996): The human biliary epithelial cell plasma membrane antigen in primary biliary cirrhosis: pyruvate dehydrogenase X? Gastroenterology, 113, 1727-1733.
34. KAPLAN M. (1996): Primary biliary cirrhosis. N. Engl. J. Med., 335, 1570-1580.
35. KIM Y. and GUM J. (1995): Diversity of mucin genes, structure, function, and expression. Gastroenterology, 109, 999-1013.
36. KOGA H., SAKISAKA S., OHISHI M., SATA M. and NATIKAWA K. (1997): Nuclear DNA fragmentation and expression of Bcl-2 in primary biliary cirrhosis. Hepatology, 25, 1077-1084.
37. KRAMS S., VAN DE WATER J., COPPEL R., ESQUIVEL C., ROBERT J., ANSARI A. and GERSHWIN M. (1990): Analysis of hepatic T lymphocyte and immunoglobulin deposits in patients with primary biliary cirrhosis. Hepatology, 12, 306-313.
38. KUROKI T., SEKI S., KAWAKITA N., NAKATANI K., HISA T. and KITADA T. (1996): Expression of antigens related to apoptosis and cell proliferation in chronic nonsuppurative destructive cholangitis in primary biliary cirrhosis. Virshows Arch., 429, 119-129.
39. LEON M., BASSENDINE M., GIBBS P., THICK M. and KIRBY J. (1997): Immunogenecity of biliary epithelium: study of the adhesive interaction with lymphocytes. Gastroenterology, 112, 968-977.
40. LEON M., KIRBY J., GIBBS P., BURT A. and BASSENDINE M. (1995): Immunogenicity of biliary epithelial cells-study of the expression of B7 molecules. J. Hepatol., 22, 591-595.
41. LEUNG P., CHUANG D., WYNN R., CHA S., DANNER D., ANSARI A., COPPEL R. and GERSHWIN M. (1995): Autoantibodies to BCOADC-E2 in patients with primary biliary cirrhosis recognize a conformational epitope. Hepatology, 22, 505-13.
42. LEUNG P., IWAYAMA T., COPPEL R. and GERSHWIN M. (1990): Site-derected mutagenesis of lysine within the immunodominant autoepitope of PDC-E2. Hepatology, 12, 1321-1328.
43. LIU Y., WENGER R., ZHAO M. and NIELSEN P. (1997): Distinct costimulatory molecules are required for the induction of effector and memory cytotoxic T lymphocytes. J. Exp. Med., 185, 251-262.
44. MANO Y., ISHII M., OKAMOTO H., IGARASHI T., KOBAYASHI K. and TOYOTA T. (1996): Effect of tumor necrosis factor alpha on intrahepatic bile duct epithelial cells of rat liver. Hepatology, 23, 1602-1607.
45. MARTINEZ-ANSO E., CASTILLO J., DIEZ J., MEDINA J. and PRIETO J. (1994): Immunohistochemical detection of chloride/ /bicarbonate anion-exchangers in human liver. Hepatology, 19, 1400-1406.
46. MARTINEZ O., VILLANUEVA J., GERSHWIN M. and KRAMS S. (1995): Cytokine patterns and cytotoxic mediators in primary biliary cirrhosis. Hepatology, 21, 113-119.
47. MATSUMOTO K., FUJII H., MICHALOPOULOS G., FUNG J. and DEMETRIS J. (1994): Human biliary epithelial cells secrete and respond to cytokines and hepatocytes growth factors in vitro: Interleukin-6, hepatocyte growth factor and epidermal growth factor promote DNA synthesis in vitro. Hepatology, 20, 376-382.
48. MEDINA J., MARTINEZ-ANSO E. and VASQUEZ J. (1997): Decreased anion exchanger 2 immunoreactivity in the liver of the patients with PBC. Hepatology, 25, 12-17.
49. MOEBIUS U., MANNS M. and HESS G. (1990): T cell receptor gene rearrangement of T lymphocytes infiltrating the liver in chronic active hepatitis B and primary biliary cirrhosis (PBC): oligoclonality of PBC-derived T cell clones. Eur. J. Immunol., 20, 889-896.
50. MOTEKI S., LEUNG P., COPPEL R., DICKSON E., KAPLAN M., MUNOZ S. and GERSHWIN M. (1996): Use of a designer triple expression hybrid clone for three different lipoyl domain for the detection of antimitochondrial autoantibodies. Hepatology, 24, 97-103.
51. NAKANUMA Y. and OHTA G. (1979): Histometric and serial section observations of the intrahepatic bile ducts in primary biliary cirrhosis. Gastroenterology, 76, 1326-1332.
52. NAKANUMA Y., TSUNEYAMA K., GERSHWIN M. and YASOSHIMA M. (1995): Pathology and immunopathology of primary biliary cirrhosis with emphasis on bile duct lesions: recent progress. Semin. Liver Dis., 15, 313-328.
53. NEUBERGER J. (1997): Primary biliary cirrhosis. Lancet, 350, 875-879.
54. NICKOWITZ R., WOZNIAK R., SCHAFFNER F. and WORMAN H. (1994): Autoantibodies against integral membrane proteins of the nuclear envelope in patients with primary biliary cirrhosis. Gastroenterology. 106, 193-199.
55. NISHIO A., VAN DE WATER J., LEUNG P., JOPLIN R., NEUBERGER J., LAKE J., BJORKLAND A., TOTTERMAN T., PETERS M., WORMAN H., ANASRI A., COPPEL R. and GERSHWIN M. (1997): Comparative study of antimitochondorial antibodies in sera and bile in primary biliary cirrhosis. Hepatology, 25, 1085-1089.
56. PORCELLI S., SEGELKE B., SUGITA M., WILSON I. and BRENNER M. (1998): The CDI family of lipid-antigen-presenting molecules. Immunol. Today, 19, 363-368.
57. QUINN J., DIAMOND A., PALMER J., BASSENDINE M., JAMES O. and YEAMAN S. (1993): Lipoylated and unlipoylated domains of human PDC-E2 as autoantigens in primary biliary cirrhosis: significance of lipoate attachment. Hepatology, 18, 1384-1391.
58. RONTOGIANNI D., GERBER H. and ZIMMERMANN A. (1994): Primary biliary cirrhosis (PBC): antigen-presenting cells differ in their distribution in early and late stage PBC and involve the ductal, but not the ductulat compartment. Histol. Histopathol., 9, 211-220.
59. SASAKI M., KONO N. and NAKANUMA Y. (1994): Neoexpression of Lewis Y antigen is a sensitive phenotypic change of the damaged intrahepatic bile ducts. Hepatology, 19, 138-144.
60. SASAKI M., NAKANUMA Y., HO S. and KIM Y. (1998): Increased MUC6 apomucin expression is a characteristic of reactive epithelium in chronic viral hepatitis. J. Pathol., 185, 191-198.
61. SASAKI M. and NAKANUMA Y. (1996): Frequent expression of MUC1 apomucin in biliary epithelial cells of damaged small bile ducts ir primary biliary cirrhosis and chronic viral hepatitis: An immunohistochemical study. Hepatology, 23, 1313-1317.
62. SCHEUER P. ( 1994): Liver biopsy interpretation. Eds 5. London, Saunders.
63. SCHWARTZ R. (1992): Costimulation of T-lymphocytes. The role of Cd28. CTLA-4, andB7/BB1 in interleukin-2 production and immunotherapy. Cell, 71, 1065-1068.
64. SHIMODA S., NAKAMURA M., ISHIBASHI H., HAYASHIDA K. and NIHO Y. (1995): HLA DRB4 0101-restricted immunodominant T cell autoepitope of pyruvate dehydrogenase complex in primary biliary cirrhosis: evidence of molecular mimicry in human autoimmune diseases. J. Exp. Med., 181, 1835-1845.
65. SHIMODA S., VAN DE WATER J., ANSARI A., NAKAMURA M., ISHIBASHI H., COPPEL R., LAKE J., KEEFFE E., ROCHE T. and GERSHWIN M. (1998): Identification and precursor frequency analysis of a common T cell epitope motif in mitochondorial autoantigens in primary biliary cirrhosis. J. Clin. Invest., 102, 1831-1840.
66. SURH C., COPPEL R. and GERSHWIN M. (1990): Structural requirement for autoreactivity on human pyruvate dehydrogenase-E2, the major autoantigen of primary biliary cirrhosis. Implication for a conformational autoepitope. J. Immunol., 144, 3367-3374.
67. SURH C., ROCHE T., DANNER D., ANSARI A., COPPEL R., PRINDIVILLE T., DICKSON E. and GERSHWIN M. (1989): Antimitochondrial autoantibodies in primary biliary cirrhosis recognize cross-reactive epitope(s) on protein X and dihydrolipoamide acetyltransferase of pyruvate dehydrogenase complex. Hepatology, 10, 127-133.
68. TERASAKI S., NAKANUMA Y., YAMAZAKI M. and UNOURA M. (1993): Eosinophilic infiltration of the liver in primary biliary cirrhosis: a morphological study. Hepatology, 17, 206-212.
69. TSAI S., LAI M. and CHEN D. (1996): Analysis of rearranged T cell receptor (TCR) V beta transcripts in livers of primary biliary cirrhosis: preferential V beta usage suggests antigen-driven selection. Clin. Exp. Immunol., 103, 99-104.
70. TSUNEYAMA K., VAN DE WATER J., LEUNG P., CHA S., NAKANUMA Y., KAPLAN M., DE LELLIS R., COPPEL R., ANSARI A. and GERSHWIN M. (1995): Abnormal expression of the E2 component of the pyruvate dehydrogenase complex on the luminal surface of biliary epithelium occurs before major histocompatibility complex class-II and BB1/B7 expression. Hepatology, 21, 1031-1037.
71. TSUNEYAMA K., YASOSHIMA M., HARADA K., HIRAMATSU K., GERSHWIN M. and NAKANUMA Y. (1998): Increased CD1d expression on small bile duct epithelium and epithelioid granuloma in livers in primary biliary cirrhosis. Hepatology, 28, 620-623.
72. VAN DE WATER J., ANSARI A., PRINDIVILLE T., COPPEL R., RICALTON N., KOTZIN B., LIU S., ROCHE T., KRAMS S., MUNOZ S. and GERSHWIN M. (1995): Heterogeneity of autoreactive T cell clones specific for the E2 component of the pyruvate dehydrogenase complex in primary biliary cirrhosis. J. Exp. Med., 181, 723-733.
73. VAN DE WATER J., ANSARI A., SURH C., COPPEL R., ROCHE T., BONKOVSKY H., KAPLAN M. and GERSHWIN M. (1991): Evidence for the targeting by 2-oxo-dehydrogenase enzymes in the T cell response of primary biliary cirrhosis. J. Immunol., 146, 89-94.
74. VAN DE WATER J., GERSHWIN M., LEUNG P., ANSARI A. and COPPEL R. (1988): The autoepitope of the 74-kD mitochondrial autoantigen of primary biliary cirrhosis corresponds to the functional site of dihydrolipoamide acetyltransferase. J. Exp. Med., 167, 1791-1799.
75. VAN DE WATER J., GERSON L., FERRELL L., LAKE J., COPPEL R., BATTS K., WIESNER R. and GERSHWIN M. (1996): Immunohistochemical evidence of disease recurrence after liver transplantation for primary biliary cirrhosis. Hepatology, 24, 1079-1084.
76. VAN DE WATER J., TURCHANY J., LEUNG P., LAKE J., MUNOZ S., SURH C., COPPEL R., ANSARI A., NAKANUMA Y. and GERSHWIN M. (1993): Molecular mimicry in primary biliary cirrhosis. Ecidence for biliary epithelial expression of a molecule cross-reactive with pyruvate dehydrogenase cimplex-E2. J. Clin. Invest., 91, 2653-2664.
77. VAN DEN OORD J., SCIOT R. and DESMET V. (1986): Expression of MHC products by normal and abnormal bile duct epithelium in primary biliary cirrhosis. J. Hepatol., 3, 310-317.
78. VYBERG M. (1993): The hepatitis-associated bile duct lesion. Liver, 13, 289-301.
79. WESIERSKA-GADEK J., HONENAUER H., HITCHMAN E. and PENNER E. (1996): Autoantibodies against nucleoporin p62 constitute a novel marker of primary biliary cirrhosis. Gastroenterology, 110, 840-847.
80. YASOSHIMA M., KONO N., SUGAHARA H., KATAYANAGI K., HARADA K. and NAKANUMA Y. (1998): Increased expression of interleukin-6 and tumor necrosis factor-α in pathologic biliary epithelial cells: in situ and culture study. Lab. Invest., 78, 89-100.
81. YEAMAN S. (1986): The mammalian 2-oxoacid dehydrogenase: a complex family. Trends Biochem. Sci., 11, 293-296.