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Passive regulation
Instability of complement components
Active regulation
Regulatory proteins (inhibit, degrade, and prevent activity)
Passive regulation
C2 = rate limiting step, C3b = unstable, C4b = unstable
C1 inhibitor
Plasma protein always present in circulation
Hereditary angioedema
Increased production of bradykinin (extreme swilling produced)
Factor H inhibitor
Binds C3b and blocks alternative pathway
Factor I inhibitor
Can terminate all complement pathways (inactivates C3b, iC3b and C4b)
Factor S inhibitor
Prevent binding to cell membranes and insertion of proteins into the membrane
Decay-accelerating factor inhibitor
CD55 = dissociates components of C3 convertase (present on peripheral blood cells)
MAC inhibitory protein inhibitor
CD59 = inhibits formation of MAC
Paroxysmal nocturnal hemoglobinuria
Immune deficiency found in RBCs (RBCs vulnerable to bystander lysis)
Complement receptor CR-1
Transport C3b-coated immune complexes to spleen and liver (stimulates phagocytosis
Complement receptor CR-2
C3b degradation (B cell stimulation)
Complement receptor CR-3 and CR-4
PRR recognizes molecular patterns on bacteria (opsonin receptors)