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. 2021 Jun 10;12:690821. doi: 10.3389/fimmu.2021.690821

Table 2.

Complement functional assays performed in this study using patient sera and mutant constructs.

Complement assays Patient 1 Patient 2 Patient 3 Positive control D279G Normal controls
D371G E601K I242L
Seruma Mutant construct Seruma Mutant construct Seruma Mutant construct Mutant construct Serum Wild-type construct
C3 deposition on glomerular endothelial cells + ND + ND + ND ND 3/10b ND
Hemolysis sheep RBCs + ND ND + ND ND 0/2 ND
Factor B binding to C3b (surface plasmon resonance) ND + ND ND + ND
Factor B degradation by factor D Degr Degr Degr Degr Degr Degr Degr Degr Degr
Hemolysis of rabbit RBCs ND + ND ND + ND
Soluble C5b-9 release from glomerular endothelial cells ND + ND ND + ND

aSerum from Patients 1 and 2 was taken during eculizumab treatment. Serum from Patient 3 was taken before the start of eculizumab treatment. b3/10 indicates positive result in 3 of 10 controls. +, complement activation detected; -, complement activation was not detected; ND, Not done; RBCs, red blood cells; Degr, degradation detected.