Download MendeleyThree-dimensional structure of the major autoantigen in primary biliary cirrhosis.
Howard, M.J., Fuller, C., Broadhurst, R.W., Perham, R.N., Tang, J.G., Quinn, J., Diamond, A.G., Yeaman, S.J.(1998) Gastroenterology 115: 139-146
- PubMed: 9649469
- DOI: https://doi.org/10.1016/s0016-5085(98)70375-0
- Primary Citation of Related Structures:
1FYC - PubMed Abstract:
Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by the presence of antimitochondrial autoantibodies in patients' serum. The major autoantigen, recognized by antibodies from > 95% of patients with PBC, has been identified as the E2 component (E2p) of the pyruvate dehydrogenase multienzyme complex. Immunodominant sites on E2p have been localized to the inner of the two lipoyl domains, where the essential cofactor lipoic acid is attached covalently. The aim of this study was to determine the three-dimensional structure of the inner lipoyl domain of human E2p.
Organizational Affiliation:
Department of Biochemistry, Cambridge Centre for Molecular Recognition, University of Cambridge, England.
