Complement Cascade Regulators Flashcards
Regulatory protein of the classical and lectin pathways.
It prevents the complement cascade from spiraling out of control, which could damage the host’s cells and tissues.
C1-INH (C1 INHIBITOR)
Detaches C1r and C1s from C1q (Inhibits the recognition unit of the classical pathway).
Inactivates MASP-2 (Inhibits the recognition unit of the lectin pathway)
C1-INH acts as a serine protease inhibitor
this genetic condition, a deficiency dysfunction in C1-INH leads to unchecked complement activation. causing episodes of severe swelling due to the overproduction of vasoactive peptides.
Hereditary angioedema (HAE)
Is a serine protease that inactivates complement components
C3b and C4b in the presence of:
• C4BP (C4-binding protein)
• CR1 (complement receptor 1)
• MCP (membrane cofactor protein)
• DAF (Decay-accelerating factor)
Factor I
DECAY ACCELERATING FACTOR (DAF)
CD55
is capable of dissociating both classical and alternative pathway C3 convertases.
DAF
Displaces Bb and C2a from C3b and C4b, respectively
It can bind to both C3b and C4b in a manner similar to CR1.
DECAY ACCELERATING FACTOR (DAF)
It does not prevent initial binding of either C2 or Factor B to the cell but can rapidly dissociate both from their binding sites, thus preventing the assembly of an active C3 convertase.
DECAY ACCELERATING FACTOR (DAF)
Protects host cells from bystander lysis
DECAY ACCELERATING FACTOR (DAF)
• Principal soluble regulator of the alternative pathway
FACTOR H
• Mode of action:
1._______ competes with factor B (B) for binding to spontaneously (hydrolytically)
activated C3b.
2. ____dissociates any C3bBb complexes that form on self-cell surfaces.
3. ______is a cofactor with factor | (FI), enabling cleavage of C3b. The resulting C3bi loses enzymatic activity but is still an opsonin.
Factor H (FH)
Factor H
Factor H
acts by binding to the fluid-phase C5b-7 complex, preventing it from attaching to nearby cell membranes.
S-PROTEIN (VITRONECTIN)
• S protein
• Inhibits C9 polymerization.
S-PROTEIN (VITRONECTIN)
• S protein
MEMBRANE INHIBITOR
OF REACTIVE LYSIS (MIRL)
“
Cb59
” It targets a later stage of MAC
assembly.
MEMBRANE INHIBITOR
OF REACTIVE LYSIS (MIRL)
“
Cb59