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Erschienen in: Journal of Gastroenterology 1/2011

01.01.2011 | International Forum

Primary biliary cirrhosis and autoimmune hepatitis: apotopes and epitopes

verfasst von: Ana Lleo, Shinji Shimoda, Hiromi Ishibashi, M. Eric Gershwin

Erschienen in: Journal of Gastroenterology | Sonderheft 1/2011

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Abstract

Autoimmune liver diseases (ALDs) represent a wide spectrum of chronic inflammatory diseases that are characterized by an immune-mediated attack against either hepatocytes (in the case of autoimmune hepatitis types 1 and 2, AIH-1, 2) or cholangiocytes (in primary biliary cirrhosis, PBC). PBC is considered a model autoimmune disease due to the homogeneity of patients, the high specificity of antimitochondrial antibodies (AMAs), and the specificity of biliary epithelial cell (BEC) destruction. It ensues from a multi-lineage loss of tolerance to the E2 component of the pyruvate dehydrogenase complex (PDC-E2). One of the major unanswered questions in the pathogenesis of PBC is the specificity of small intrahepatic bile duct attack while PDC-E2 is present in mitochondria of nucleated cells. Recent findings suggest that the apoptosis of BECs may be of considerable importance for understanding PBC, and that they are more than simply an innocent victim of an immune attack. Rather, they attract immune attack by virtue of the unique biochemical mechanisms by which they handle PDC-E2. The role of apoptotic cells in AIH is not well defined, but advances in the study of autoreactive T cells stem mostly from AIH type 2, where the main autoantigen (CYP2D6) is known, enabling the characterization of antigen-specific immune responses. This review article is intended to provide a critical overview of current evidence on tissue specificity in ALDs, as well as the characteristics of the relevant epitopes and apotopes and their biological and clinical significance.
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Metadaten
Titel
Primary biliary cirrhosis and autoimmune hepatitis: apotopes and epitopes
verfasst von
Ana Lleo
Shinji Shimoda
Hiromi Ishibashi
M. Eric Gershwin
Publikationsdatum
01.01.2011
Verlag
Springer Japan
Erschienen in
Journal of Gastroenterology / Ausgabe Sonderheft 1/2011
Print ISSN: 0944-1174
Elektronische ISSN: 1435-5922
DOI
https://doi.org/10.1007/s00535-010-0303-8

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