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Functionally active complement proteins C6 and C7 detected in C6- and C7-deficient individuals
R. WÜRZNER,
A. ORREN, P. POTTER,
B. P. MORGAN,
D. PONARD,
P. SPÄTH,
M. BRAI, M. SCHULZE,
L. HAPPE,
O. GÖTZE,
Corresponding Author
R. WÜRZNER
Departments of Immunology, University of Göttingen, Germany
Reinhard Würzner, MD, MRC Molecular Im munopathology Unit, University of Cambridge, Hills Road, GB-Cambridge CB2 2QH, UK.Search for more papers by this authorP. POTTER
Clinical Science and Immunology, University of Cape Town, South Africa
Search for more papers by this authorB. P. MORGAN
Medical Biochemistry, University of Wales, Cardiff, UK
Search for more papers by this authorD. PONARD
Centre Hospitalier Regional, University of Grenoble, France
Search for more papers by this authorP. SPÄTH
Blood Transfusion Service, Swiss Red Cross, Berne, Switzerland
Search for more papers by this authorM. SCHULZE
Departments of Immunology, University of Göttingen, Germany
Search for more papers by this authorL. HAPPE
Departments of Immunology, University of Göttingen, Germany
Search for more papers by this authorO. GÖTZE
Departments of Immunology, University of Göttingen, Germany
Search for more papers by this authorR. WÜRZNER,
A. ORREN, P. POTTER,
B. P. MORGAN,
D. PONARD,
P. SPÄTH,
M. BRAI, M. SCHULZE,
L. HAPPE,
O. GÖTZE,
Corresponding Author
R. WÜRZNER
Departments of Immunology, University of Göttingen, Germany
Reinhard Würzner, MD, MRC Molecular Im munopathology Unit, University of Cambridge, Hills Road, GB-Cambridge CB2 2QH, UK.Search for more papers by this authorP. POTTER
Clinical Science and Immunology, University of Cape Town, South Africa
Search for more papers by this authorB. P. MORGAN
Medical Biochemistry, University of Wales, Cardiff, UK
Search for more papers by this authorD. PONARD
Centre Hospitalier Regional, University of Grenoble, France
Search for more papers by this authorP. SPÄTH
Blood Transfusion Service, Swiss Red Cross, Berne, Switzerland
Search for more papers by this authorM. SCHULZE
Departments of Immunology, University of Göttingen, Germany
Search for more papers by this authorL. HAPPE
Departments of Immunology, University of Göttingen, Germany
Search for more papers by this authorO. GÖTZE
Departments of Immunology, University of Göttingen, Germany
Search for more papers by this authorSUMMARY
Two sensitive sandwich ELISAs based on monoclonal antibodies directed to native C6 and C7 allowed the detection and quantitation of these complement proteins in 20 out of 37 serum samples from individuals who had previously been classified as deficient in these proteins as assessed by immunochemical and/or functional assays. Furthermore, serum from four C6-deficient and one combined C6-/C7-deficient individual showed an increase in the terminal complement complex (TCC) and a decrease in native C6 and C7 after complement activation as assayed by specific ELISAs. Despite their (incomplete) deficiencies, these individuals therefore possess functionally active terminal complement proteins with respect to their ability to generate the TCC. As these individuals have no history of a susceptibility to neisserial infections, even low concentrations of functionally active C6 and C7 may provide sufficient protection against those micro-organisms whose destruction requires TCC formation.
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