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- Title
Demonstration of PDC-E1 subunits as major antigens in the complement-fixing fraction M4 and re-evaluation of PDC-E1-specific antibodies in PBC patients.
- Authors
Berg, Christoph P.; Stein, Gerburg M.; Klein, Reinhild; Pascu, Maria; Berg, Thomas; Kammer, Winfried; Priemer, Martin; Nordheim, Alfred; Schulze-Osthoff, Klaus; Gregor, Michael; Wesselborg, Sebastian; Berg, Peter A.
- Abstract
Primary biliary cirrhosis (PBC) is characterized by the presence of antimitochondrial antibodies (AMA). Autoantibodies specific for the mitochondrial M4 antigen can be detected by a complement fixation test (CFT) but not by immunoblotting. The aim of this study was to elucidate the identity of the M4 antigen. Patients and methods: M4 proteins were purified by affinity chromatography using IgG fractions of PBC marker sera being CFT positive ( n=5) or negative ( n=5) and identified by Western blotting, silver staining and sequence analysis. Further, a cohort of 57 PBC patients was tested for the reactivity to M4 and pyruvate dehydrogenase complex (PDC). Results: Two AMA patterns of the marker sera were visualized: CFT-positive sera were defined as PDC-E2+/E1+ and the CFT-negative sera as PDC-E2+/E1−. The major proteins in the M4 fraction could be related to the PDC-E1 subunits. A clear-cut association between anti-M4 reactivity in the CFT and the reactivity to both PDC subunits could also be documented in the cohort of 57 PBC patients showing anti-PDC-E1α and E1β antibodies at a frequency of 74% and 67%. Conclusions: CFT reactivity against M4 antigens could be preferentially identified as a reaction against PDC-E1. As PDC-E1 subunits as compared with PDC-E2 lack lipoyl-binding sites, they probably have to be considered as an independent and important target.
- Subjects
CIRRHOSIS of the liver; ANTIGENS; IMMUNOGLOBULINS; IMMUNOBLOTTING; SILVER staining (Microscopy)
- Publication
Liver International, 2006, Vol 26, Issue 7, p846
- ISSN
1478-3223
- Publication type
Article
- DOI
10.1111/j.1478-3231.2006.01303.x