Loading…
The immunology of primary biliary cirrhosis: the end of the beginning?
SUMMARY The chronic liver disease primary biliary cirrhosis (PBC) is characterised by autoreactive B‐cell and T‐cell responses directed against mitochondrial antigens. In recent years these responses have been extensively characterised and the principal PBC associated autoantigen identified as pyruv...
Saved in:
| Published in: | Clinical and experimental immunology 2002-08, Vol.129 (2), p.191-197 |
|---|---|
| Main Authors: | , , |
| Format: | Article |
| Language: | English |
| Subjects: | |
| Citations: | Items that this one cites Items that cite this one |
| Online Access: | Get full text |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| cited_by | cdi_FETCH-LOGICAL-c5588-371d246b571c65c3f89c40faf2b685919f2787a0f6f5d6e67998585eb4a213c83 |
|---|---|
| cites | cdi_FETCH-LOGICAL-c5588-371d246b571c65c3f89c40faf2b685919f2787a0f6f5d6e67998585eb4a213c83 |
| container_end_page | 197 |
| container_issue | 2 |
| container_start_page | 191 |
| container_title | Clinical and experimental immunology |
| container_volume | 129 |
| creator | PALMER, J. M. KIRBY, J. A. JONES, D. E. J. |
| description | SUMMARY
The chronic liver disease primary biliary cirrhosis (PBC) is characterised by autoreactive B‐cell and T‐cell responses directed against mitochondrial antigens. In recent years these responses have been extensively characterised and the principal PBC associated autoantigen identified as pyruvate dehydrogenase complex (PDC). The identification of anti‐PDC responses (present in over 95% of PDC patients) has given rise to important questions pertinent to our understanding of the pathogenesis of PBC. What specific role to anti‐PDC responses play in target cell damage? How and why does immune tolerance break down to as highly conserved and ubiquitously expressed self‐antigen as PDC? Why does breakdown in tolerance to an antigen present in all nucleated cells result in damage restricted to the intra‐hepatic bile ducts? In attempting to answer these key questions we have, in this review, proposed a unifying hypothesis for the pathogenesis of PBC. |
| doi_str_mv | 10.1046/j.1365-2249.2002.01948.x |
| format | article |
| fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_1906441</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>71978170</sourcerecordid><originalsourceid>FETCH-LOGICAL-c5588-371d246b571c65c3f89c40faf2b685919f2787a0f6f5d6e67998585eb4a213c83</originalsourceid><addsrcrecordid>eNqNkUtvEzEUhS0EoqHwF9AICXYz-P1AoqiKWqhUiU1ZWx7HThxN7GJnoPn39ZBRC2xgda91v3t87ANAg2CHIOXvtx0inLUYU9VhCHEHkaKyu3sCFg-Dp2ABIVStqhsn4EUp23rknOPn4ARhxBkUZAEubzauCbvdGNOQ1ocm-eY2h53Jh6YPQ5iqDTlvUgnlQ7OvsIuriZra3q1DjCGuP70Ez7wZins111Pw7fLiZvmlvf76-Wp5ft1axqRsiUArTHnPBLKcWeKlshR643HPJVNIeSykMNBzz1bccaGUZJK5nhqMiJXkFJwddW_HfudW1sV9NoOeLetkgv5zEsNGr9MPjRTklKIq8G4WyOn76Mpe70KxbhhMdGksWiAlJBLwnyCSlBKKJ8U3f4HbNOZYf6FeyqUUhOEKySNkcyolO_9gGUE9Raq3ekpOT8npKVL9K1J9V1df__7kx8U5wwq8nQFTrBl8NtGG8sgRiZkStHIfj9zPMLjDfxvQy4urqSP3Tyi7gA</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>196887352</pqid></control><display><type>article</type><title>The immunology of primary biliary cirrhosis: the end of the beginning?</title><source>Open Access: PubMed Central</source><creator>PALMER, J. M. ; KIRBY, J. A. ; JONES, D. E. J.</creator><creatorcontrib>PALMER, J. M. ; KIRBY, J. A. ; JONES, D. E. J.</creatorcontrib><description>SUMMARY
The chronic liver disease primary biliary cirrhosis (PBC) is characterised by autoreactive B‐cell and T‐cell responses directed against mitochondrial antigens. In recent years these responses have been extensively characterised and the principal PBC associated autoantigen identified as pyruvate dehydrogenase complex (PDC). The identification of anti‐PDC responses (present in over 95% of PDC patients) has given rise to important questions pertinent to our understanding of the pathogenesis of PBC. What specific role to anti‐PDC responses play in target cell damage? How and why does immune tolerance break down to as highly conserved and ubiquitously expressed self‐antigen as PDC? Why does breakdown in tolerance to an antigen present in all nucleated cells result in damage restricted to the intra‐hepatic bile ducts? In attempting to answer these key questions we have, in this review, proposed a unifying hypothesis for the pathogenesis of PBC.</description><identifier>ISSN: 0009-9104</identifier><identifier>EISSN: 1365-2249</identifier><identifier>DOI: 10.1046/j.1365-2249.2002.01948.x</identifier><identifier>PMID: 12165073</identifier><identifier>CODEN: CEXIAL</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Science Ltd</publisher><subject>animal models of disease apoptosis autoimmune disease liver cirrhosis ; Animals ; Autoantibodies - biosynthesis ; Biliary Tract - immunology ; biliary molecular mimicry xenobiotic ; Biological and medical sciences ; Epithelial Cells - immunology ; Gastroenterology. Liver. Pancreas. Abdomen ; Humans ; Immunity, Cellular ; Liver Cirrhosis, Biliary - etiology ; Liver Cirrhosis, Biliary - immunology ; Liver. Biliary tract. Portal circulation. Exocrine pancreas ; Medical sciences ; Mice ; Models, Immunological ; Other diseases. Semiology ; Pyruvate Dehydrogenase Complex - immunology ; Review ; Self Tolerance ; T-Lymphocytes - immunology</subject><ispartof>Clinical and experimental immunology, 2002-08, Vol.129 (2), p.191-197</ispartof><rights>2003 INIST-CNRS</rights><rights>Copyright Blackwell Scientific Publications Ltd. Aug 2002</rights><rights>2002 Blackwell Science Ltd 2002</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c5588-371d246b571c65c3f89c40faf2b685919f2787a0f6f5d6e67998585eb4a213c83</citedby><cites>FETCH-LOGICAL-c5588-371d246b571c65c3f89c40faf2b685919f2787a0f6f5d6e67998585eb4a213c83</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC1906441/pdf/$$EPDF$$P50$$Gpubmedcentral$$H</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC1906441/$$EHTML$$P50$$Gpubmedcentral$$H</linktohtml><link.rule.ids>230,314,727,780,784,885,27924,27925,53791,53793</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=13825974$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/12165073$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>PALMER, J. M.</creatorcontrib><creatorcontrib>KIRBY, J. A.</creatorcontrib><creatorcontrib>JONES, D. E. J.</creatorcontrib><title>The immunology of primary biliary cirrhosis: the end of the beginning?</title><title>Clinical and experimental immunology</title><addtitle>Clin Exp Immunol</addtitle><description>SUMMARY
The chronic liver disease primary biliary cirrhosis (PBC) is characterised by autoreactive B‐cell and T‐cell responses directed against mitochondrial antigens. In recent years these responses have been extensively characterised and the principal PBC associated autoantigen identified as pyruvate dehydrogenase complex (PDC). The identification of anti‐PDC responses (present in over 95% of PDC patients) has given rise to important questions pertinent to our understanding of the pathogenesis of PBC. What specific role to anti‐PDC responses play in target cell damage? How and why does immune tolerance break down to as highly conserved and ubiquitously expressed self‐antigen as PDC? Why does breakdown in tolerance to an antigen present in all nucleated cells result in damage restricted to the intra‐hepatic bile ducts? In attempting to answer these key questions we have, in this review, proposed a unifying hypothesis for the pathogenesis of PBC.</description><subject>animal models of disease apoptosis autoimmune disease liver cirrhosis</subject><subject>Animals</subject><subject>Autoantibodies - biosynthesis</subject><subject>Biliary Tract - immunology</subject><subject>biliary molecular mimicry xenobiotic</subject><subject>Biological and medical sciences</subject><subject>Epithelial Cells - immunology</subject><subject>Gastroenterology. Liver. Pancreas. Abdomen</subject><subject>Humans</subject><subject>Immunity, Cellular</subject><subject>Liver Cirrhosis, Biliary - etiology</subject><subject>Liver Cirrhosis, Biliary - immunology</subject><subject>Liver. Biliary tract. Portal circulation. Exocrine pancreas</subject><subject>Medical sciences</subject><subject>Mice</subject><subject>Models, Immunological</subject><subject>Other diseases. Semiology</subject><subject>Pyruvate Dehydrogenase Complex - immunology</subject><subject>Review</subject><subject>Self Tolerance</subject><subject>T-Lymphocytes - immunology</subject><issn>0009-9104</issn><issn>1365-2249</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2002</creationdate><recordtype>article</recordtype><recordid>eNqNkUtvEzEUhS0EoqHwF9AICXYz-P1AoqiKWqhUiU1ZWx7HThxN7GJnoPn39ZBRC2xgda91v3t87ANAg2CHIOXvtx0inLUYU9VhCHEHkaKyu3sCFg-Dp2ABIVStqhsn4EUp23rknOPn4ARhxBkUZAEubzauCbvdGNOQ1ocm-eY2h53Jh6YPQ5iqDTlvUgnlQ7OvsIuriZra3q1DjCGuP70Ez7wZins111Pw7fLiZvmlvf76-Wp5ft1axqRsiUArTHnPBLKcWeKlshR643HPJVNIeSykMNBzz1bccaGUZJK5nhqMiJXkFJwddW_HfudW1sV9NoOeLetkgv5zEsNGr9MPjRTklKIq8G4WyOn76Mpe70KxbhhMdGksWiAlJBLwnyCSlBKKJ8U3f4HbNOZYf6FeyqUUhOEKySNkcyolO_9gGUE9Raq3ekpOT8npKVL9K1J9V1df__7kx8U5wwq8nQFTrBl8NtGG8sgRiZkStHIfj9zPMLjDfxvQy4urqSP3Tyi7gA</recordid><startdate>200208</startdate><enddate>200208</enddate><creator>PALMER, J. M.</creator><creator>KIRBY, J. A.</creator><creator>JONES, D. E. J.</creator><general>Blackwell Science Ltd</general><general>Blackwell</general><general>Oxford University Press</general><general>Blackwell Science Inc</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>7U9</scope><scope>H94</scope><scope>M7N</scope><scope>7X8</scope><scope>5PM</scope></search><sort><creationdate>200208</creationdate><title>The immunology of primary biliary cirrhosis: the end of the beginning?</title><author>PALMER, J. M. ; KIRBY, J. A. ; JONES, D. E. J.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5588-371d246b571c65c3f89c40faf2b685919f2787a0f6f5d6e67998585eb4a213c83</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2002</creationdate><topic>animal models of disease apoptosis autoimmune disease liver cirrhosis</topic><topic>Animals</topic><topic>Autoantibodies - biosynthesis</topic><topic>Biliary Tract - immunology</topic><topic>biliary molecular mimicry xenobiotic</topic><topic>Biological and medical sciences</topic><topic>Epithelial Cells - immunology</topic><topic>Gastroenterology. Liver. Pancreas. Abdomen</topic><topic>Humans</topic><topic>Immunity, Cellular</topic><topic>Liver Cirrhosis, Biliary - etiology</topic><topic>Liver Cirrhosis, Biliary - immunology</topic><topic>Liver. Biliary tract. Portal circulation. Exocrine pancreas</topic><topic>Medical sciences</topic><topic>Mice</topic><topic>Models, Immunological</topic><topic>Other diseases. Semiology</topic><topic>Pyruvate Dehydrogenase Complex - immunology</topic><topic>Review</topic><topic>Self Tolerance</topic><topic>T-Lymphocytes - immunology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>PALMER, J. M.</creatorcontrib><creatorcontrib>KIRBY, J. A.</creatorcontrib><creatorcontrib>JONES, D. E. J.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>Algology Mycology and Protozoology Abstracts (Microbiology C)</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><jtitle>Clinical and experimental immunology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>PALMER, J. M.</au><au>KIRBY, J. A.</au><au>JONES, D. E. J.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>The immunology of primary biliary cirrhosis: the end of the beginning?</atitle><jtitle>Clinical and experimental immunology</jtitle><addtitle>Clin Exp Immunol</addtitle><date>2002-08</date><risdate>2002</risdate><volume>129</volume><issue>2</issue><spage>191</spage><epage>197</epage><pages>191-197</pages><issn>0009-9104</issn><eissn>1365-2249</eissn><coden>CEXIAL</coden><abstract>SUMMARY
The chronic liver disease primary biliary cirrhosis (PBC) is characterised by autoreactive B‐cell and T‐cell responses directed against mitochondrial antigens. In recent years these responses have been extensively characterised and the principal PBC associated autoantigen identified as pyruvate dehydrogenase complex (PDC). The identification of anti‐PDC responses (present in over 95% of PDC patients) has given rise to important questions pertinent to our understanding of the pathogenesis of PBC. What specific role to anti‐PDC responses play in target cell damage? How and why does immune tolerance break down to as highly conserved and ubiquitously expressed self‐antigen as PDC? Why does breakdown in tolerance to an antigen present in all nucleated cells result in damage restricted to the intra‐hepatic bile ducts? In attempting to answer these key questions we have, in this review, proposed a unifying hypothesis for the pathogenesis of PBC.</abstract><cop>Oxford, UK</cop><pub>Blackwell Science Ltd</pub><pmid>12165073</pmid><doi>10.1046/j.1365-2249.2002.01948.x</doi><tpages>7</tpages><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0009-9104 |
| ispartof | Clinical and experimental immunology, 2002-08, Vol.129 (2), p.191-197 |
| issn | 0009-9104 1365-2249 |
| language | eng |
| recordid | cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_1906441 |
| source | Open Access: PubMed Central |
| subjects | animal models of disease apoptosis autoimmune disease liver cirrhosis Animals Autoantibodies - biosynthesis Biliary Tract - immunology biliary molecular mimicry xenobiotic Biological and medical sciences Epithelial Cells - immunology Gastroenterology. Liver. Pancreas. Abdomen Humans Immunity, Cellular Liver Cirrhosis, Biliary - etiology Liver Cirrhosis, Biliary - immunology Liver. Biliary tract. Portal circulation. Exocrine pancreas Medical sciences Mice Models, Immunological Other diseases. Semiology Pyruvate Dehydrogenase Complex - immunology Review Self Tolerance T-Lymphocytes - immunology |
| title | The immunology of primary biliary cirrhosis: the end of the beginning? |
| url | http://sfxeu10.hosted.exlibrisgroup.com/loughborough?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2024-12-29T13%3A03%3A46IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_pubme&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=The%20immunology%20of%20primary%20biliary%20cirrhosis:%20the%20end%20of%20the%20beginning?&rft.jtitle=Clinical%20and%20experimental%20immunology&rft.au=PALMER,%20J.%20M.&rft.date=2002-08&rft.volume=129&rft.issue=2&rft.spage=191&rft.epage=197&rft.pages=191-197&rft.issn=0009-9104&rft.eissn=1365-2249&rft.coden=CEXIAL&rft_id=info:doi/10.1046/j.1365-2249.2002.01948.x&rft_dat=%3Cproquest_pubme%3E71978170%3C/proquest_pubme%3E%3Cgrp_id%3Ecdi_FETCH-LOGICAL-c5588-371d246b571c65c3f89c40faf2b685919f2787a0f6f5d6e67998585eb4a213c83%3C/grp_id%3E%3Coa%3E%3C/oa%3E%3Curl%3E%3C/url%3E&rft_id=info:oai/&rft_pqid=196887352&rft_id=info:pmid/12165073&rfr_iscdi=true |