Figure 1.
The complement cascade. A simplified schematic of the alternative, lectin and classical pathways of complement. Each of these initiating pathways leads to the generation of C3 convertases (C3bBb or C4b2a) and C5 convertases (C3bBbC3b or C4b2aC3b). C5 convertases cleave C5 and to trigger the generation of membrane attack complex or C5b-9. The presence of C4Nef and/or C3Nef stabilizes C3 convertases, resulting in hyperactivity of the alternative and terminal pathways.(MASP, mannose-associated serine protease; P, properdin; MAC, membrane attack complex; C3Nef, C3 nephritic factor; C4Nef, C4 nephritic factor; FHAA, Factor H autoantibody; FBAA, Factor B autoantibody).