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Autoantibodies to BCOADC-E2 in patients with primary biliary cirrhosis recognize a conformational epitope

✍ Scribed by Patrick S. C. Leung; David T. Chuang; R. Max Wynn; Dr. Sanghoon Cha; Dean J. Danner; Aftab Ansari; Ross L. Coppel; M. Eric Gershwin

Publisher: John Wiley and Sons
Year: 1995
Tongue: English
Weight: 1020 KB
Volume: 22
Category: Article
ISSN: 0270-9139
DOI: 10.1002/hep.1840220220

No coin nor oath required. For personal study only.

✦ Synopsis

AFTAB ANSAKI,' Ross L. cOk>i)E12,' AND M. ERIC GERSHWIN' Primary biliary cirrhosis (PBC) is an autoimmune disease of liver associated with a unique serologic response to mitochondrial autoantigens. Many of the autoantigens recognized by autoantibodies in PBC are members of the 2-0x0-acid dehydrogenase complex. The two major autoantigens are the E2 component of the pyruvate dehydrogenase complex (PDC-E2) and the E2 component of the branched chain 2-0x0-acid dehydrogenase complex (BCOADC-E2). The autoantibody response to PDC-E2 has been mapped to one immunodominant epitope, which consists of both linear and conformational components. The presence of a single immunodominant epitope in PDC-E2 is unusual when contrasted to the immune response to autoantigens in other human autoimmune diseases. We have mapped the epitope recognized by antimitochondrial autoantibodies (AMA) specific to BCOADC-E2 in patients with PBC by taking advantage of the full-length bovine BCOADC-E2 complementary DNA (cDNA) and a series of expression clones spanning the entire molecule. Reactivity to the various expression clones was studied by immunoblotting, enzyme-linked immunosorbent assay (ELISA), as well as selective absorption of patient sera by expressed protein fragments. Autoantibodies to BCOADC-E2 map within peptides spanning amino acid residues 1 to 227 of the mature protein; our data demonstrate that the epitope is dependent on conformation and includes the lipoic Abbreviations: AhlA, antimitocliondnal antibodies: PM', primary hiliary PDC-E2. pyruvate dehydrogenase complex E2: H('OAIX-EZ. chain 2-oxo-acid dehydrogenase complex E2; cDNA. complenientary DNA: IPTG. isopropylthiogalactosid~; SDS-PAGE, sodium dodticyl siilrate piilyacrylamidr gel clectrophoresis: ELISA. enzyme-linked iinnlui1~isorbent assay

From the 'Division of lilieumatology. N l e r ~ and Clinical Ininiunology.

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