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. Author manuscript; available in PMC: 2019 Aug 30.
Published in final edited form as: Xenotransplantation. 2019 Apr 29;26(4):e12517. doi: 10.1111/xen.12517

Table 2:

Regulators of the complement system10, 1518

Regulator Target Function Pathway Location
C1-INH C1qrs, MASPs Inactivates C1r and C1s, MASP-1 and MASP-2 CP/LP Fluid
Small mannose-binding lectin-ficolin-associated protein (sMAP, MAP19) MBL Competes with MAPS1–3 LP Fluid
Mannose-binding lectin-ficolin-associated protein-1 (MAP-1) MBL/ficolins Competes with MAPS LP Fluid
C4BP C4b Inhibits C3 convertase CP/LP Fluid
Factor H C3b, Factor B, Bb Blocks the formation of C3 convertases CP/LP/AP Membrane and fluid
Factor H-related proteins 1–5 C3b, Factor H Competes with factor H CP/AP Membrane and fluid
Factor I C3b,C4b Degrades C3b or C4b with cofactor CP/LP/AP Fluid
CR1 C3b,C4b Inhibits C3/C5 convertase CP/LP/AP Membrane
CD46 (MCP) C3b,C4b Inhibits C3/C5 convertase.
Cofactor for Factor I.
CP/LP/AP Membrane
CD55 (DAF) C3b,C4b, C3convertase C3/C5 convertase decay CP/LP/AP Membrane
Signal Regulatory Protein α (SIRPα and family) MAC-1(CR3) Inhibits phagocytosis CP/LP/AP Membrane
Crry C3/C5 convertase Inhibits C3/C5 convertase CP/LP/AP Membrane
vWF C3b Helps with C3b cleavage CP/LP/AP Fluid
Thrombomodulin (CD141) C3a, C3b, C4b, C5a C3/C5 convertase decay. Anaphylatoxin inhibitor CP/LP/AP Membrane and fluid
Plasmin(ogen) C3, C3b, C3d, and C5 C3/C5 convertase decay CP/LP/AP Membrane and fluid
Carboxypeptidase N, R, B (thrombin-activatable fibrinolysis inhibitor) C3a, C5a Anaphylatoxin inhibitor CP/LP/AP Fluid
Cartilage oligomeric matrix protein (thrombospondin 5) C1q, MBL Inhibits C3 convertase CP/LP Fluid
C3, Properdin Activates AP AP Fluid
CUB (structural motif consistsof C1r/C1s, Uegf, Bmp1) and sushi multiple domains 1 (CSMD1) C3b, C4b Cofactor of Factor I? CP/LP? Membrane
Sushi domain-containing protein 4 (SUSD4) C1q, MBL, C3b? Inhibits C3 convertase CP/LP/AP Membrane and fluid (?)
Clusterin (apolipoprotein J, SP-40) C5b-8 Inhibits MAC formation TP Fluid
Vitronectin (VN, S-protein, S40) C5b-9 Inhibits MAC formation TP Fluid
CD59 (HRF20, protctin) C8, C9 Inhibits MAC formation TP Membrane

Abbreviations: AP = Alternative pathway; C1-INH = C1 inhibitor; C4BP = C4-binding protein; CR1 = Complement receptor 1 (CD35); CP = Classical pathway; Crry = Complement receptor-related protein, expressed in mouse and rat; DAF = CD55, decay-accelerating factor; LP = Lectin pathway; MASP = Mannose-binding lectin-associated serine proteases; MBL = Mannose-binding lectin; MCP = CD46, membrane cofactor protein; TP = Terminal pathway; vWF = von Willebrand factor.