Autoantibodies against nuclear proteins are not always but rather frequently present in sera of patients with primary biliary cirrhosis (PBC). The specificity and diagnostic value of these autoantibodies for PBC have only recently become clear through cloning of the cDNA of some of the corresponding autoantigens which allowed the establishment of immunological assays with recombinant autoantigens expressed in E. coli and eukaryotic cells. In this report we summarize primarily the knowledge on the structure and putative function of two nuclear autoantigens, the Sp100 and PML proteins, which are present in so-called nuclear dots (NDs) and against which autoantibodies are present in a subpopulation of PBC patients. Furthermore, the type of autoimmune response (epitope specificity and immunoglobulin class) against both the Sp100 and PML proteins and the specificity of the anti-Sp100 and anti-PML autoantibodies for PBC patients and patients with other autoimmune diseases is reviewed. Current knowledge clearly indicates that determination of anti-Sp100 and anti-PML autoantibodies substantially improves diagnosis of PBC as these autoantibodies are highly specific for this disease even when autoantibodies against mitochondrial antigens, a hallmark of most PBC patients, are not found. The type of autoimmune response against the Sp100 and PML proteins also provides some dikes about possible mechanisms which lead to autoantigenicity of both proteins.
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Department of Pathology, University Hospital of Heraklion
3. Department of Gastroenterology, University Hospital of Herak-lionLiver Research Laboratory, Medical School, University of Crete
Maria Tzardi
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Argyro Voumvouraki
Aikaterini Afgoustaki
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机构:Liver Research Laboratory, Medical School, University of Crete
Aikaterini Afgoustaki
Meri Koulentaki
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机构:Liver Research Laboratory, Medical School, University of Crete
机构:Chaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
Agmon-Levin, Nancy
Shapira, Yinon
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机构:Chaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
Shapira, Yinon
Selmi, Carlo
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Univ Milan, Dept Internal Med, IRCCS, Ist Clin Humanitas, I-20122 Milan, ItalyChaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
Selmi, Carlo
Barzilai, Ori
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机构:Chaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
Barzilai, Ori
Ram, Maya
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机构:Chaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
Ram, Maya
Szyper-Kravitz, Martine
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机构:Chaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
Szyper-Kravitz, Martine
Sella, Sara
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机构:Chaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
Sella, Sara
Katz, Bat-sheva Porat
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Hebrew Univ Jerusalem, Fac Agr Food & Environm Qual Sci, IL-91905 Jerusalem, IsraelChaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
Katz, Bat-sheva Porat
Youinou, Pierre
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机构:
Univ Milan, Dept Internal Med, IRCCS, Ist Clin Humanitas, I-20122 Milan, Italy
Brest Univ, Med Sch Hosp, Immunol Lab, Brest, FranceChaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
Youinou, Pierre
Renaudineau, Yves
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Univ Milan, Dept Internal Med, IRCCS, Ist Clin Humanitas, I-20122 Milan, Italy
Brest Univ, Med Sch Hosp, Immunol Lab, Brest, FranceChaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
Renaudineau, Yves
Larida, Bruno
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机构:Chaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
Larida, Bruno
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Invernizzi, Pietro
Gershwin, M. Eric
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Univ Calif Davis, Sch Med, Div Rheumatol Allergy & Clin Immunol, Davis, CA 95616 USAChaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
Gershwin, M. Eric
Shoenfeld, Yehuda
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Chaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
Tel Aviv Univ, Sackler Fac Med, Incumbent Laura Schwarz Kip Chair Res Autoimmune, Tel Aviv, IsraelChaim Sheba Med Ctr, Dept Med B, WM Keck Autoimmune Dis Ctr, IL-52621 Tel Hashomer, Israel
机构:
Depart ment of Medicine and Research Laboratory of Internal Medicine, Medical School,University of Thessaly,41110 Larissa,GreeceDepart ment of Medicine and Research Laboratory of Internal Medicine, Medical School,University of Thessaly,41110 Larissa,Greece
Theodoros A Zografos
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Nikolaos Gatselis
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Kalliopi Zachou
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Christos Liaskos
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Stella Gabeta
George K Koukoulis
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Department of Pathology,Medical School,University of Thessaly,41110 Larissa,GreeceDepart ment of Medicine and Research Laboratory of Internal Medicine, Medical School,University of Thessaly,41110 Larissa,Greece
George K Koukoulis
George N Dalekos
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Depart ment of Medicine and Research Laboratory of Internal Medicine, Medical School,University of Thessaly,41110 Larissa,GreeceDepart ment of Medicine and Research Laboratory of Internal Medicine, Medical School,University of Thessaly,41110 Larissa,Greece