Table 1.
A: Soluble complement regulatory factors | |
---|---|
REGULATOR | FUNCTION |
C1 inhibitor | Serine protease that targets the C1s/C1r, inhibiting activation of C4 and C2 [19] |
Factor H | Modulates C3b formation by acting as a co-factor for Factor I and by accelerating the decay of the C3 convertases [20] |
Factor I (C3b /C4b inactivator) | Decreases complement activation by cleaving C3b and C4b when complexed with co-factors such as CD46 [21,22] |
C4 Binding Protein (C4BP) | A co-factor for Factor I, binds C4b increasing proteolytic accessibility [23] |
Vitronectin (S40) | Inhibits the terminal cascade and formation of the MAC; may have other roles in regulation of disease responses [24–26] |
Clusterin (Apo J) | Similar to vitronectin, inhibits the formation of the MAC and may have other functions [26] |
B: Membrane-bound complement regulatory factors | |
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REGULATOR | FUNCTION |
CD35 (Complement receptor 1, CR1) | Decay accelerating factor for C3/C5 convertases, facilitates phagocytosis of cells with complement activated, co-factor for Factor I, fixes complement immune complexes on erythrocytes, has limited tissue distribution in humans [27] |
CD46 (Membrane cofactor protein, MCP) | Cofactor for factor I, regulator of T-cell differentiation and apoptosis, widely expressed in humans [16,28,29] |
CD55 (Decay accelerating factor, DAF) | Inhibits formation and accelerates decay of C3 convertases [30–32] |
CD59 (MAC inhibitory protein, MAC-IP, 20 kDa homologous restriction factor, HRF20) | Inhibits formation of the MAC by binding C5b/8 complex and interfering with insertion of C9 [33,34] |