 |
PDBsum entry 1fyc
|
|
|
|
References listed in PDB file
|
 |
|
Key reference
|
|
|
Title
|
|
Three-Dimensional structure of the major autoantigen in primary biliary cirrhosis.
|
|
|
Authors
|
|
M.J.Howard,
C.Fuller,
R.W.Broadhurst,
R.N.Perham,
J.G.Tang,
J.Quinn,
A.G.Diamond,
S.J.Yeaman.
|
|
|
Ref.
|
|
Gastroenterology, 1998,
115,
139-146.
|
|
|
PubMed id
|
|
|
|
|
Note In the PDB file this reference is
annotated as "TO BE PUBLISHED".
The citation details given above were identified by an automated
search of PubMed on title and author
names, giving a
percentage match of
0%.
|
|
|
|
Abstract
|
|
|
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 covalently. The aim of this study was to
determine the three-dimensional structure of the inner lipoyl domain of human
E2p. METHODS: The domain was expressed in Escherichia coli; after purification,
its structure was analyzed using nuclear magnetic resonance spectroscopy.
RESULTS: The structure of the lipoyl domain from human E2p was determined, and
the implications of the structure for autoimmune recognition were assessed.
CONCLUSIONS: Knowledge of the structure further defines the major epitope and
may help in the design of antigen-specific immunotherapy for treatment of PBC.
|
|
|
|
|
|
|
