Mitochondrial antigens as targets of cellular and humoral auto-immunity in primary biliary cirrhosis |
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| Authors: | Yasunori Ichiki Carlo Selmi Shinji Shimoda Hiromi Ishibashi Stuart C Gordon M Eric Gershwin |
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| Affiliation: | (1) Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, TB192 Davis, CA;(2) Medicine and Biosystemic Science, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, 812-8582 Fukuoka, Japan;(3) Clinical Research Center, Nagasaki Medical Center, Kubara 2-1001-1, Omura, 856-8562 Nagasaki, Japan;(4) Division of Gastroenterology/Hepatology, William Beaumont Hospital, Royal Oak, MI |
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| Abstract: | Several factors point toward an auto-immune pathogenesis for primary biliary cirrhosis (PBC), mostly based on the presence
of serum auto-antibodies to mitochondrial antigens (AMAs) and autoreactive T cells (both helper and cytotoxic). Interestingly,
epitopes recognized by AMA and T-cell clones are located within overlapping areas of the antigens. Moreover, a role for an
imbalance in cytokine pattern and for natural-killer lymphocytes has also been proposed. Despite several experimental reports,
no clear evidence is available regarding the interaction of these factors leading to bile duct destruction. This article reviews
the current reports regarding the auto-immune reaction against mitochondrial auto-antigens in PBC. |
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| Keywords: | Auto-immunity auto-antibody auto-epitope primary biliary cirrhosis 2-oxo-acid dehydrogenase complex pyruvate dehydrogenase complex |
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