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Formation of a hemolytically active cellular intermediate by the interaction between properdin factors B and D and the activated third component of complement.

The present studies demonstrate that the factor B-dependent C3 convertase can be affixed to an erythrocyte by use of an intermediate bearing C3b and that this convertase brings the hemolytic reaction to completion with an efficiency comparable to that of classical convertase. The evidence that the EAC43 intermediate was lysed by a new pathway includes requirements for factors B and D and cell-bound C3b for subsequent lysis by the terminal components, C3-C9. The linear stoichiometry of the effective molecule titrations of C3b and factor B, and the first-order kinetics displayed by the generation and decay of the factor B-dependent hemolytic site are characteristics consistent with the one-hit theory as initially developed for the classical complement system. The use of hemolytically active cellular intermediates to examine the reactions occurring with C3b and factors B and D has allowed extension of the one-hit theory to this molecular sequence, development of effective molecule titrations, recognition of the analogies to the functional characteristics of the classical C3 convertase, and discrimination of the probable mechanism of terminal complement activation from reactive lysis.

Complement System Proteins↗

Properdin factor B (Bf) polymorphism in Norway.

Bf phenotype distribution and Bf allele frequencies in samples of the Norwegian population and the Lappish minority of Norway are presented. Bfs in Norwegians was found to be 0.817, while it was 0.888 in Lapps. The difference is statistically significant. Possible explanations of this difference are discussed. It is noted that Bf allele frequencies of Norwegians are similar to those of Germans, while differing from frequencies found in US whites.

Alleles↗

Frequency of complement third component (C3) and properdin factor (BF) phenotypes in patients with various clinical manifestations of HBV infection.

Four groups of children were tested for the distribution of C3 and BF phenotypes: HBV-infected patients with Gianotti-Crosti syndrome, with CAH and with glomerulonephritis, and healthy children. The frequency of the phenotype C3F was significantly higher in children with Gianotti-Crosti syndrome in comparison with healthy children. The frequency of the phenotype BFS was significantly higher in patients with glomerulonephritis than in individuals with CAH. The difference between the frequency of the BFS phenotype in glomerulonephritis patients and that in healthy subjects neared significance. We suggest that the carriers of these phenotypes are characterized by susceptibility to some immune complex diseases associated with HBV infection.

Acrodermatitis↗

C3 and C5-cleaving properdin enzymes formed on zymosan incubated with human serum: the decay and the regeneration of the enzymes.

Incubation os zymosan (Z) with normal human serum led to the formation of ZXhu, which had the abilities to cleave C3 and C5. Kinetic studies on ZXhu formation revealed that the amount of inactivated C5 by ZXhu, when expressed in site-forming unit (SFU), was much greater than that of inactivated C3. When ZXhu having limited C3 and C5-cleaving was incubated at 37 degrees C it decayed with a first order reaction completely in 120 min. At any stage of decay of ZXhu, the activities could be restored by the addition of both B and D, but not by the addition of B or D alone. Not D but B could be bound to the completely decayed ZXhu, and activation of bound B by D led to the regeneration of ZXhu.

Beta-Globulins↗