A case of deficiency of the seventh component of complement in man. Biological properties of a C7-deficient serum and description of a C7-inactivating principle.
The serum of a 13-year-old healthy boy was found to be deficient in whole complement activity. The seventh component of complement could not be detected by functional assays whereas the titres of the other components were found within the normal range. An attempt to detect C7 by immunodiffusion against an antiserum to human C7 also failed. The functions of C1-C6 were normal with respect to opsonizing activity, immune adherence, and the ability to generate chemotactic activity. However, those functions that require the whole complement sequence such as bactericidal and haemolytic activity were found to be absent. Furthermore, the occurrence of a C7-inactivating principle was demonstrated in the C7-deficient serum. This principle inactivated C7 both in the fluid phase and in its cell-bound state. Some physicochemical parameters of the inactivator are described and its possible nature is discussed.
D000293:Adolescent; D001770:Blood Bactericidal Activity; D002633:Chemotaxis; D002850:Chromatography, Gel; D003184:Complement C7 / Q000037:antagonists & inhibitors / Q000172:deficiency*; D003165:Complement System Proteins / Q000172:deficiency*; D006461:Hemolysis; D006801:Humans; D007104:Immune Adherence Reaction; D005779:Immunodiffusion; D007700:Kinetics; D008297:Male; D009895:Opsonin Proteins; D010587:Phagocytosis; D013696:Temperature; D013997:Time Factors; D015054:Zymosan
D003184:Complement C7; D009895:Opsonin Proteins; D003165:Complement System Proteins; D015054:Zymosan