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Characterization of soluble terminal complement complex assembled in C8beta-deficient plasma and serum
K Høgåsen1, T E Mollnes, W Nürnberger
1Institute of Immunology and Rheumatology, University of Oslo, Norway.
Scandinavian Journal of Immunology
|September 22, 1998
Summary
Complement component C8 is crucial for haemolytic activity. Studies show soluble terminal complement complex (TCC) can form even in C8beta-deficient sera, indicating C8beta is not always essential for TCC assembly.
Area of Science:
- Immunology
- Complement System Biology
Background:
- Complement component C8 (C8) is essential for the terminal pathway of the complement system, mediating haemolytic activity.
- Genetic deficiencies in C8 subunits (alpha-gamma or beta) severely impair complement-mediated lysis.
Purpose of the Study:
- To investigate the formation and structure of soluble terminal complement complex (TCC) in sera deficient in C8 subunits.
- To determine the role of C8beta in TCC assembly.
Main Methods:
- Activation of C8-deficient sera with cobra venom factor (CVF).
- Quantification of TCC using enzyme immunoassay.
- Purification of TCC by affinity chromatography.
- Analysis of TCC composition via immunoblotting.
Main Results:
- C8alpha-gamma and C8beta-deficient sera showed significantly reduced TCC formation compared to normal serum.
- Purified TCC from C8beta-deficient serum contained minimal to no C8beta, but normal levels of other terminal components.
- Experiments with purified components confirmed C8alpha-gamma can facilitate TCC assembly with limited or absent C8beta.
Conclusions:
- Soluble TCC can assemble in C8beta-deficient sera, even with very low or absent C8beta.
- The C8alpha-gamma subunit plays a significant role in initiating TCC formation, independent of substantial C8beta presence.