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. 2018 Apr 4;9:612. doi: 10.3389/fimmu.2018.00612

Figure 6.

Figure 6

Assessment of convertase activity in a family with complement factor B (FB) mutation and atypical hemolytic uremic syndrome (aHUS). (A) Pedigree with carriers of the FB p.Lys323Glu mutation and/or aHUS. No genetic information of this variation was available for I. 2 who is deceased. (B) Analysis of convertase stability in available samples from seven family members. The sera were tested mixed 1:1 with normal human serum (NHS) to a final concentration of 5%. Data for each test sample were obtained from three independent experiments; means are given with error bars showing SDs. Statistical analysis for test sera compared with NHS from t = 20 to t = 60 as calculated using two-way analysis of variance is given: *P < 0.05, ***P < 0.001, ns not significant. (C) Assessment of the presence of convertase-stabilizing factors in the immunoglobulin (Ig) fraction of aHUS patient III. 6. Purified Igs from patient plasma or pooled normal human plasma (NHP) were added in an equal volume to 5% NHS. Data were collected from three independent experiments; means are given with error bars showing SDs. (B,C) Heat-inactivated NHS (ΔNHS) and an NHS sample from which guinea pig serum were omitted during the second part of the assay (no step 2) served as negative controls for the first and second steps, respectively. Hemolysis levels are given as percentage of full lysis of erythrocytes in water.