Three-dimensional structure of the major autoantigen in primary biliary cirrhosis.

  title={Three-dimensional structure of the major autoantigen in primary biliary cirrhosis.},
  author={Mark J. Howard and C. Fuller and R. William Broadhurst and Richard N. Perham and Jong Tang and Janet Quinn and Austin G. Diamond and Stephen J. Yeaman},
  volume={115 1},
BACKGROUND & AIMS 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… CONTINUE READING