6533b85ffe1ef96bd12c1e2b

RESEARCH PRODUCT

Functionally active complement proteins C6 and C7 detected in C6- and C7-deficient individuals

B.p. MorganPaul C. PotterP.j. SpäthDenise PonardOtto GötzeR. WürznerAnn OrrenM. BraiM. SchulzeL. Happe

subject

Blood Bactericidal Activitymedicine.drug_classImmunoblottingImmunologyEnzyme-Linked Immunosorbent AssayBiologyMonoclonal antibodyComplement Hemolytic Activity AssaySpecimen Handling03 medical and health sciences0302 clinical medicineTerminal complement complexImmunopathologymedicineHumansImmunology and AllergyComplement ActivationVolume concentration030304 developmental biology0303 health sciencesTemperatureZymosanAntibodies MonoclonalComplement deficiencyComplement C9Serum samplesmedicine.diseaseMolecular biologyComplement C7Complement C63. Good healthComplement (complexity)Complement systemImmunologyElectrophoresis Polyacrylamide GelResearch Article030215 immunology

description

SUMMARYTwo 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.

https://doi.org/10.1111/j.1365-2249.1991.tb05656.x