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1. Full Text IL‐2 receptor α−/− mice and the development of primary biliary cirrhosis
by Wakabayashi, Kanji and Lian, Zhe‐Xiong and Moritoki, Yuki and Lan, Ruth Y and Tsuneyama, Koichi and Chuang, Ya‐Hui and Yang, Guo‐Xiang and Ridgway, William and Ueno, Yoshiyuki and Ansari, Aftab A and Coppel, Ross L and Mackay, Ian R and Gershwin, M. Eric
Hepatology, ISSN 0270-9139, 11/2006, Volume 44, Issue 5, pp. 1240 - 1249
Recently, we identified a child born with a genetic deficiency of IL‐2 receptor α (IL‐2Rα, CD25) expression who had several clinical manifestations of primary... 
ANTIMITOCHONDRIAL ANTIBODIES | B-CELLS | IMMUNE-RESPONSE | AUTOIMMUNITY | TOLERANCE | LIVER | DISEASE | REGULATORY T-CELLS | GASTROENTEROLOGY & HEPATOLOGY | HYPER-IGM | LYMPHOCYTES | Interleukin-2 Receptor alpha Subunit - deficiency | Mice, Inbred C57BL | Male | Mitochondria - immunology | T-Lymphocytes, Regulatory - pathology | Phenotype | Animals | Flow Cytometry | Liver Cirrhosis, Biliary - immunology | Immunoglobulins - blood | Liver Cirrhosis, Biliary - etiology | Lymphocyte Count | Female | Mice | Cytokines - blood | Disease Models, Animal | Interleukin-2 Receptor alpha Subunit - immunology | Index Medicus
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2. Full Text Anti-mitochondrial antibodies and primary biliary cirrhosis in TGF-βreceptor II dominant-negative mice
by Oertelt, Sabine and Lian, Zhe-Xiong and Cheng, Chun-Mei and Chuang, Ya-Hui and Padgett, Kerstien A and He, Xiao-Song and Ridgway, William M and Ansari, Aftab A and Coppel, Ross L and Li, Ming O and Flavell, Richard A and Kronenberg, Mitchell and Mackay, Ian R and Gershwin, M. Eric
Journal of Immunology, ISSN 0022-1767, 08/2006, Volume 177, Issue 3, pp. 1655 - 1660
Primary biliary cirrhosis (PBC) is an autoimmune disease of the liver, characterized by lymphocytic infiltrates in portal tracts, selective destruction of... 
IMMUNE-RESPONSE | EPITHELIAL-CELLS | DISEASE | LIVER | REGULATORY T-CELLS | IMMUNOLOGY | EXPRESSION | FOXP3 | Liver - pathology | Receptors, Transforming Growth Factor beta - genetics | CD8-Positive T-Lymphocytes - pathology | Species Specificity | Protein-Serine-Threonine Kinases | Male | Mitochondria - immunology | Autoantibodies - biosynthesis | CD4-Positive T-Lymphocytes - pathology | Cell Movement - genetics | Cell Movement - immunology | Liver - immunology | Liver Cirrhosis, Biliary - immunology | Receptor, Transforming Growth Factor-beta Type II | Female | Lymphocyte Subsets - pathology | Mice, Inbred C57BL | Mice, Transgenic | Liver Cirrhosis, Biliary - pathology | Liver Cirrhosis, Biliary - genetics | Mice, Knockout | Animals | Mice | Bile Ducts - immunology | Bile Ducts - pathology | Cytokines - biosynthesis | Receptors, Transforming Growth Factor beta - deficiency | Index Medicus | Abridged Index Medicus
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3. Full Text CXCR5+ CD4+ T follicular helper cells participate in the pathogenesis of primary biliary cirrhosis
by Wang, Lifeng and Sun, Ying and Zhang, Zheng and Jia, Yiqiong and Zou, Zhengsheng and Ding, Jinbiao and Li, Yuanyuan and Xu, Xiangsheng and Jin, Lei and Yang, Tao and Li, Zhiwei and Sun, Yanling and Zhang, Ji‐yuan and Lv, Sa and Chen, Liming and Li, Baosen and Gershwin, M. Eric and Wang, Fu‐Sheng
Hepatology, ISSN 0270-9139, 02/2015, Volume 61, Issue 2, pp. 627 - 638
There is increasing interest in the role of T follicular helper (Tfh) cells in autoimmunity from the perspective of both their role in breach of tolerance and... 
B-CELL | PROGNOSIS | FREQUENCY | EXPANSION | DISEASE | BIOCHEMICAL RESPONSE | MITOCHONDRIAL ANTIGEN | IDENTIFICATION | GASTROENTEROLOGY & HEPATOLOGY | NONSUPPURATIVE DESTRUCTIVE CHOLANGITIS | SEVERITY | Receptors, CXCR5 - metabolism | Humans | Middle Aged | Liver Cirrhosis, Biliary - drug therapy | B-Lymphocytes - physiology | Male | Case-Control Studies | Cholagogues and Choleretics - therapeutic use | Interleukins - metabolism | Ursodeoxycholic Acid - therapeutic use | Young Adult | T-Lymphocytes, Helper-Inducer - physiology | Liver Cirrhosis, Biliary - immunology | Adult | Female | Autoimmune diseases | Liver cirrhosis | Index Medicus | Primary biliary cirrhosis | plasma B cells | ursodeoxycholic acid | T follicular helper cells
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4. Full Text Genome-wide association study identifies 12 new susceptibility loci for primary biliary cirrhosis
by Mells, George F and Floyd, James A B and Morley, Katherine I and Cordell, Heather J and Franklin, Christopher S and Shin, So-Youn and Heneghan, Michael A and Neuberger, James M and Donaldson, Peter T and Day, Darren B and Ducker, Samantha J and Muriithi, Agnes W and Wheater, Elizabeth F and Hammond, Christopher J and Dawwas, Muhammad F and UK PBC Consortium, PBC Consortium and Wellcome Trust Case Control Consortium 3, Trust Case Control Consortium 3 and Jones, David E and Peltonen, Leena and Alexander, Graeme J and Sandford, Richard N and Anderson, Carl A and The UK PBC Consortium and The Wellcome Trust Case Control Co and UK PBC Consortium and Wellcome Trust Case Control Consortium 3 and The Wellcome Trust Case Control Consortium 3
Nature genetics, ISSN 1061-4036, 2011, Volume 43, Issue 4, pp. 329 - 332
In addition to the HLA locus, six genetic risk factors for primary biliary cirrhosis (PBC) have been identified in recent genome-wide association studies... 
PATHOGENESIS | VARIANTS | GENE | HLA | GENETICS & HEREDITY | TOOL | B7-1 Antigen - genetics | Humans | Immunity, Innate - genetics | Databases, Genetic | Male | NF-kappa B p50 Subunit - genetics | Death Domain Receptor Signaling Adaptor Proteins - genetics | Lectins, C-Type - genetics | Case-Control Studies | Adaptive Immunity - genetics | Receptors, Interleukin-7 - genetics | Liver Cirrhosis, Biliary - immunology | Female | Monosaccharide Transport Proteins - genetics | Genetic Predisposition to Disease | Genome-Wide Association Study | Guanine Nucleotide Exchange Factors - genetics | Receptors, CXCR5 - genetics | Risk Factors | Liver Cirrhosis, Biliary - genetics | Receptors, Tumor Necrosis Factor, Type I - genetics | Linkage Disequilibrium | STAT4 Transcription Factor - genetics | Polymorphism, Single Nucleotide | Cohort Studies | Care and treatment | Disease susceptibility | Genetic aspects | Biliary cirrhosis | Single nucleotide polymorphisms | Research | Risk factors | Medical research | Biomedical research | Disease | Health services | Genes | Genomes | Liver cirrhosis | Index Medicus
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5. Full Text Ongoing activation of autoantigen‐specific B cells in primary biliary cirrhosis
by Zhang, Jun and Zhang, Weici and Leung, Patrick S.C and Bowlus, Christopher L and Dhaliwal, Sandeep and Coppel, Ross L and Ansari, Aftab A and Yang, Guo‐Xiang and Wang, Jinjun and Kenny, Thomas P and He, Xiao‐Song and Mackay, Ian R and Gershwin, M. Eric
Hepatology, ISSN 0270-9139, 11/2014, Volume 60, Issue 5, pp. 1708 - 1716
The serologic hallmark of primary biliary cirrhosis (PBC), the antimitochondrial response to the E2 component of the pyruvate dehydrogenase complex (PDC-E2),... 
PYRUVATE-DEHYDROGENASE | ANTIBODIES | AUTOIMMUNITY | INFLAMMATION | DISEASE | E2 SUBUNIT | CHOLANGITIS | SECRETING CELLS | GASTROENTEROLOGY & HEPATOLOGY | AUTOEPITOPE | PLASMA-CELLS | Antibody Specificity | Antibody-Producing Cells - metabolism | Receptors, CCR10 - metabolism | Dihydrolipoyllysine-Residue Acetyltransferase - immunology | Humans | Middle Aged | Male | Case-Control Studies | Young Adult | B-Lymphocytes - immunology | Liver Cirrhosis, Biliary - immunology | Receptors, CXCR - metabolism | Autoantigens - immunology | Aged, 80 and over | Adult | Female | Aged | Enzymes | Medical research | Antigens | Immunoglobulins | Index Medicus | PDC-E2 | antibody cross-reactivity | Plasmablast | autoreactive B cell | autoantigen-specific
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6. Full Text Interaction between Toll‐like receptors and natural killer cells in the destruction of bile ducts in primary biliary cirrhosis
by Shimoda, Shinji and Harada, Kenichi and Niiro, Hiroaki and Shirabe, Ken and Taketomi, Akinobu and Maehara, Yoshihiko and Tsuneyama, Koichi and Nakanuma, Yasuni and Leung, Patrick and Ansari, Aftab A and Gershwin, M. Eric and Akashi, Koichi
Hepatology, ISSN 0270-9139, 04/2011, Volume 53, Issue 4, pp. 1270 - 1281
Primary biliary cirrhosis (PBC) is characterized by chronic nonsuppurative destructive cholangitis (CNSDC) associated with destruction of small bile ducts.... 
CROSS-TALK | RESPONSES | ACTIVATION | NK CELLS | EPITHELIAL-CELLS | AUTOIMMUNITY | DENDRITIC CELL | LIVER-DISEASE | GASTROENTEROLOGY & HEPATOLOGY | T-CELLS | I INTERFERON | Poly I-C - pharmacology | Interferon-alpha - biosynthesis | Epithelial Cells - drug effects | Humans | Toll-Like Receptors - immunology | Toll-Like Receptor 3 - immunology | Toll-Like Receptor 4 - immunology | Liver Cirrhosis, Biliary - pathology | Toll-Like Receptor 3 - metabolism | Toll-Like Receptor 4 - metabolism | Monocytes - drug effects | Immunity, Innate - immunology | Liver Cirrhosis, Biliary - immunology | Lipopolysaccharides - pharmacology | Killer Cells, Natural - immunology | Liver - cytology | Monocytes - physiology | Bile Ducts - immunology | Bile Ducts - pathology | Cytotoxicity | Ligands | Liver cirrhosis | Index Medicus | IFN-α | natural killer cells | monocytes | toll-like receptors | biliary epithelial cells | primary biliary cirrhosis
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7. Full Text Anti‐gp210 and anti‐centromere antibodies are different risk factors for the progression of primary biliary cirrhosis
by Nakamura, Minoru and Kondo, Hisayoshi and Mori, Tsuyoshi and Komori, Atsumasa and Matsuyama, Mutsumi and Ito, Masahiro and Takii, Yasushi and Koyabu, Makiko and Yokoyama, Terufumi and Migita, Kiyoshi and Daikoku, Manabu and Abiru, Seigo and Yatsuhashi, Hiroshi and Takezaki, Eiichi and Masaki, Naohiko and Sugi, Kazuhiro and Honda, Koichi and Adachi, Hiroshi and Nishi, Hidehiro and Watanabe, Yukio and Nakamura, Yoko and Shimada, Masaaki and Komatsu, Tatsuji and Saito, Akira and Saoshiro, Takeo and Harada, Hideharu and Sodeyama, Takeshi and Hayashi, Shigeki and Masumoto, Akihide and Sando, Takehiro and Yamamoto, Tetsuo and Sakai, Hironori and Kobayashi, Masakazu and Muro, Toyokichi and Koga, Michiaki and Shums, Zakera and Norman, Gary L and Ishibashi, Hiromi
Hepatology, ISSN 0270-9139, 01/2007, Volume 45, Issue 1, pp. 118 - 127
The predictive role of antinuclear antibodies (ANAs) remains elusive in the long‐term outcome of primary biliary cirrhosis (PBC). The progression of PBC was... 
AUTOANTIBODIES | URSODEOXYCHOLIC ACID | MOLECULAR MIMICRY | ANTINUCLEAR ANTIBODIES | PREVALENCE | SYSTEMIC-SCLEROSIS | GASTROENTEROLOGY & HEPATOLOGY | EXPRESSION | T-CELLS | CLINICAL-SIGNIFICANCE | NUCLEAR AUTOANTIGENS | Liver Failure - etiology | Multivariate Analysis | Prospective Studies | Antibodies, Antinuclear - immunology | Humans | Middle Aged | Male | Esophageal and Gastric Varices - etiology | Hypertension, Portal - etiology | Liver Cirrhosis, Biliary - immunology | Antibodies, Antinuclear - blood | Chromatin - immunology | Aged, 80 and over | Nuclear Pore Complex Proteins - immunology | Adult | Female | Retrospective Studies | Risk Factors | Nuclear Proteins - immunology | Liver Cirrhosis, Biliary - classification | Disease Progression | Autoantigens - immunology | Centromere - immunology | Aged | Liver Cirrhosis, Biliary - complications | Antigens, Nuclear - immunology | Index Medicus
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8. Full Text Liver‐targeted and peripheral blood alterations of regulatory T cells in primary biliary cirrhosis
by Lan, Ruth Y and Cheng, Chunmei and Lian, Zhe‐Xiong and Tsuneyama, Koichi and Yang, Guo‐Xiang and Moritoki, Yuki and Chuang, Ya‐Hui and Nakamura, Takafumi and Saito, Shigeru and Shimoda, Shinji and Tanaka, Atsushi and Bowlus, Christopher L and Takano, Yasuo and Ansari, Aftab A and Coppel, Ross L and Gershwin, M. Eric
Hepatology, ISSN 0270-9139, 04/2006, Volume 43, Issue 4, pp. 729 - 737
CD4 + CD25 high regulatory T cells (Tregs) play a critical role in self‐tolerance, as seen in murine autoimmunity. Studies on Tregs in human autoimmunity have... 
RESPONSES | ACTIVATION | DENDRITIC CELLS | SELF | GASTROENTEROLOGY & HEPATOLOGY | AUTOIMMUNE-DISEASE | FOXP3 | IMMUNOLOGICAL-TOLERANCE | CUTTING EDGE | Autoimmunity | Receptors, Tumor Necrosis Factor - metabolism | Severity of Illness Index | T-Lymphocytes, Regulatory - metabolism | Liver Cirrhosis, Biliary - physiopathology | Humans | CD4-Positive T-Lymphocytes - metabolism | Glucocorticoid-Induced TNFR-Related Protein | Receptors, Interleukin-2 - metabolism | Hepatitis, Autoimmune - immunology | CD4 Lymphocyte Count | Receptors, Nerve Growth Factor - metabolism | Liver Cirrhosis, Biliary - pathology | CD4-Positive T-Lymphocytes - pathology | Hepatitis C, Chronic - blood | Case-Control Studies | T-Lymphocytes, Regulatory - pathology | T-Lymphocytes, Regulatory - immunology | Liver - immunology | Liver Cirrhosis, Biliary - immunology | Forkhead Transcription Factors - metabolism | Hepatitis C, Chronic - immunology | Hepatitis, Autoimmune - blood | Liver Cirrhosis, Biliary - blood | Index Medicus
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9. Full Text Alterations and correlations of the gut microbiome, metabolism and immunity in patients with primary biliary cirrhosis
by Lv, Long‐Xian and Fang, Dai‐Qiong and Shi, Ding and Chen, De‐Ying and Yan, Ren and Zhu, Yi‐Xin and Chen, Yan‐Fei and Shao, Li and Guo, Fei‐Fei and Wu, Wen‐Rui and Li, Ang and Shi, Hai‐Yan and Jiang, Xia‐Wei and Jiang, Hui‐Yong and Xiao, Yong‐Hong and Zheng, Shu‐Sen and Li, Lan‐Juan
Environmental Microbiology, ISSN 1462-2912, 07/2016, Volume 18, Issue 7, pp. 2272 - 2286
We selected 42 early‐stage primary biliary cirrhosis (PBC) patients and 30 healthy controls (HC). Metagenomic sequencing of the 16S rRNA gene was used to... 
FERMENTATION | MICROBIOLOGY | INFECTIONS | PROFILE | STARCH | Metagenomics | Humans | Middle Aged | Gastrointestinal Microbiome | Gastrointestinal Tract - microbiology | Male | Bacteria - genetics | Gastrointestinal Tract - metabolism | Feces - microbiology | Gastrointestinal Tract - immunology | Bacteria - isolation & purification | Liver Cirrhosis, Biliary - immunology | Bacteria - classification | Female | Liver Cirrhosis, Biliary - metabolism | Liver Cirrhosis, Biliary - microbiology | Care and treatment | Microbiota (Symbiotic organisms) | RNA | Liver | Physiological aspects | Bacteria | Biliary cirrhosis | Metabolism | Genes | Index Medicus
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10. Full Text IgG4-related retroperitoneal fibrosis overlapping with primary biliary cirrhosis and primary Sjogren's syndrome A case report
by Huang, X and Lu, B and Li, M and Fan, YH and Zhang, L
MEDICINE, ISSN 0025-7974, 06/2018, Volume 97, Issue 26, pp. e11303 - e11303
Rationale: IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder which is characterized by elevated levels of serum IgG4 and infiltration of... 
primary Sjogren's syndrome | DIAGNOSIS | MEDICINE, GENERAL & INTERNAL | primary biliary cirrhosis | SYSTEMIC-DISEASE | INVOLVEMENT | TREATMENT RESPONSE | SERUM IGG4 LEVELS | COHORT | SCLEROSING PANCREATITIS | immunoglobulin G4-related retroperitoneal fibrosis | REGISTRY | Retroperitoneal Fibrosis - drug therapy | Immunoglobulin G - blood | Antibodies, Antinuclear - immunology | Humans | Sjogren's Syndrome - immunology | Male | Cholagogues and Choleretics - therapeutic use |