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Primary structure of the human M2 mitochondrial autoantigen of primary biliary cirrhosis: dihydrolipoamide acetyltransferase.
Open accessAbstract
Primary biliary cirrhosis is a chronic, destructive autoimmune liver disease of humans. Patient sera are characterized by a high frequency (greater than 95%) of autoantibodies to a Mr 70,000 mitochondrial antigen, a component of the M2 antigen complex. We have identified a human cDNA clone encoding the complete amino acid sequence of this autoantigen. The predicted structure has significant similarity with the dihydrolipoamide acetyltransferase (EC 2.3.1.12) of the Escherichia coli pyruvate dehydrogenase multienzyme complex. The human sequence preserves the Glu-Thr-Asp-Lys-Ala motif of the lipoyl-binding site and has two potential binding sites. Expressed fragments of the cDNA react strongly with sera from patients with primary biliary cirrhosis but not with sera from patients with autoimmune chronic active hepatitis or sera from healthy subjects.
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Citations
Cited by
Ye, Q., & Worman, H. J. (1996) · Journal of Biological Chemistry · doi:10.1074/jbc.271.25.14653 · Cited by 406
Züchner, D., Sternsdorf, T., Szostecki, C., Heathcote, E. J., Cauch-Dudek, K., & Will, H. (1997) · Hepatology · doi:10.1002/hep.510260506 · Cited by 404
The immunobiology and pathophysiology of primary biliary cirrhosis
Hirschfield, G. M., & Gershwin, M. E. (2013) · Annual Review of Pathology: Mechanisms of Disease · doi:10.1146/annurev-pathol-020712-164014 · Cited by 315
Self-citationCaforio, A. L., Grazzini, M., Mann, J. M., Keeling, P. J., Bottazzo, G. F., McKenna, W. J., & Schiaffino, S. (1992) · Circulation · doi:10.1161/01.cir.85.5.1734 · Cited by 315
Pathogenesis of cholestatic liver disease and therapeutic approaches
Hirschfield, G. M., Heathcote, E. J., & Gershwin, M. E. (2010) · Gastroenterology · doi:10.1053/j.gastro.2010.09.004 · Cited by 287
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Metadata
- Authors
- Coppel, R. L., McNeilage, L. J., Surh, C. D., Van de Water, J., Spithill, T. W., Whittingham, S., & Gershwin, M. E.
- Venue
- Proceedings of the National Academy of Sciences
- Published
- 1988-10
- Volume
- 85
- Issue
- 19
- Pages
- 7317-7321
- ISSN
- 0027-8424 (ISSN-L)
- 1091-6490
- OpenAlex
- W2093304786
- Cited by
- 254
- References
- 0
- FWCI
- 20.09
- Type
- journal article
- Language
- en
- Open access
- green
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- CoChat. (2026). Citation check: Primary structure of the human M2 mitochondrial autoantigen of primary biliary cirrhosis: dihydrolipoamide acetyltransferase. https://app.cochat.ai/papers/10.1073/pnas.85.19.7317
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- Coppel, R. L., McNeilage, L. J., Surh, C. D., Van de Water, J., Spithill, T. W., Whittingham, S., & Gershwin, M. E. (1988). Primary structure of the human M2 mitochondrial autoantigen of primary biliary cirrhosis: dihydrolipoamide acetyltransferase. Proceedings of the National Academy of Sciences. https://doi.org/10.1073/pnas.85.19.7317
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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