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8.10.08
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normally, C1INH prevents spontaneous activation of the classical pathway
how would this spontaneous activation occur? well, IgM sticks onto RBCs transiently and is able to fix complement. the C1 INH is what prevents the cascade from being triggered by IgM
usually, at low levels of antigen, C1INH wins and removes C1r, C1s from active C1 complex
however, at high levels of antigen, C1INH is overwhelmed and complement pathway is activated
without C1INH, active C1 complex is never deactivated and as a consequence C2 and C4 are cleaved
furthermore, C1 can also be activated spontaneously, a process aided by plasmin
Category: Pathology Notes
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