Abstract
Mitochondrial autoantigens and their B and T cell autoepitopes have been well defined in primary biliary cirrhosis (PBC). However, the relationships of the antimitochondrial antibodies and the mechanisms of bile duct destruction in PBC remain an enigma. The serological hallmark of PBC remains the presence of antibodies to mitochondria, particularly to the E2 component of the pyruvate dehydrogenase complex (PDC-E2). However, several mechanisms may now be proposed which may explain the immune-mediated bile duct damage in PBC. These include the possible role of T cell-mediated cytotoxicity as well as the interaction between the IgA class of antimitochondrial antibodies and the mitochondrial autoantigens. A prominent feature in this discussion is the highly directed and specific immune response to the mitochondrial antigens, including PDC-E2 as well as other members of the 2-oxo-acid dehydrogenase complexes. Ultimately, the mechanisms that lead to this immune reaction should provide data on other questions in PBC, including the reasons for female predominance, the absence of PBC in children and the relative ineffectiveness of immunosuppressive agents.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 535-547 |
| Number of pages | 13 |
| Journal | Bailliere's Best Practice and Research in Clinical Gastroenterology |
| Volume | 14 |
| Issue number | 4 |
| DOIs | |
| State | Published - 2000 |
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Keywords
- 2-oxo-acid dehydrogenase complex
- Autoepitopes
- Primary biliary cirrhosis
ASJC Scopus subject areas
- Gastroenterology
Cite this
The pyruvate dehydrogenase complex as a target autoantigen in primary biliary cirrhosis. / Nishio, Akiyoshi; Coppel, Ross; Ishibashi, Hiromi; Gershwin, M. Eric.
In: Bailliere's Best Practice and Research in Clinical Gastroenterology, Vol. 14, No. 4, 2000, p. 535-547.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - The pyruvate dehydrogenase complex as a target autoantigen in primary biliary cirrhosis
AU - Nishio, Akiyoshi
AU - Coppel, Ross
AU - Ishibashi, Hiromi
AU - Gershwin, M. Eric
PY - 2000
Y1 - 2000
N2 - Mitochondrial autoantigens and their B and T cell autoepitopes have been well defined in primary biliary cirrhosis (PBC). However, the relationships of the antimitochondrial antibodies and the mechanisms of bile duct destruction in PBC remain an enigma. The serological hallmark of PBC remains the presence of antibodies to mitochondria, particularly to the E2 component of the pyruvate dehydrogenase complex (PDC-E2). However, several mechanisms may now be proposed which may explain the immune-mediated bile duct damage in PBC. These include the possible role of T cell-mediated cytotoxicity as well as the interaction between the IgA class of antimitochondrial antibodies and the mitochondrial autoantigens. A prominent feature in this discussion is the highly directed and specific immune response to the mitochondrial antigens, including PDC-E2 as well as other members of the 2-oxo-acid dehydrogenase complexes. Ultimately, the mechanisms that lead to this immune reaction should provide data on other questions in PBC, including the reasons for female predominance, the absence of PBC in children and the relative ineffectiveness of immunosuppressive agents.
AB - Mitochondrial autoantigens and their B and T cell autoepitopes have been well defined in primary biliary cirrhosis (PBC). However, the relationships of the antimitochondrial antibodies and the mechanisms of bile duct destruction in PBC remain an enigma. The serological hallmark of PBC remains the presence of antibodies to mitochondria, particularly to the E2 component of the pyruvate dehydrogenase complex (PDC-E2). However, several mechanisms may now be proposed which may explain the immune-mediated bile duct damage in PBC. These include the possible role of T cell-mediated cytotoxicity as well as the interaction between the IgA class of antimitochondrial antibodies and the mitochondrial autoantigens. A prominent feature in this discussion is the highly directed and specific immune response to the mitochondrial antigens, including PDC-E2 as well as other members of the 2-oxo-acid dehydrogenase complexes. Ultimately, the mechanisms that lead to this immune reaction should provide data on other questions in PBC, including the reasons for female predominance, the absence of PBC in children and the relative ineffectiveness of immunosuppressive agents.
KW - 2-oxo-acid dehydrogenase complex
KW - Autoepitopes
KW - Primary biliary cirrhosis
UR - http://www.scopus.com/inward/record.url?scp=0033792437&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0033792437&partnerID=8YFLogxK
U2 - 10.1053/bega.2000.0102
DO - 10.1053/bega.2000.0102
M3 - Article
C2 - 10976013
AN - SCOPUS:0033792437
VL - 14
SP - 535
EP - 547
JO - Best Practice and Research: Clinical Gastroenterology
JF - Best Practice and Research: Clinical Gastroenterology
SN - 1521-6918
IS - 4
ER -