complement system Flashcards
C3 is synthesized by the
liver
C3 is converted to
C3b
C3b role
binds to bacteria causing death
alternative pathway
C3 spontaneously converts to C3b
surfaces that bind C3b making it stable
LPS
fungal cell walls
viral envelopes
stable C3b can bind
complement protein b
__ clips B bound to C3b
complement protein D
when complement protein D clips B bound to C3b it forms
C3 convertase C3bBb
role of C3 convertase
to cleave more C3 to C3b
factor H is synthesized in
liver
factor H blocks
alternative pathway on host cells
mannose binding lectin is produced by
liver
mannose binding lectin circulates with mannose associated serine proteases and binds
surfaces with mannose
in the classical pathway C1 cleaves
C2 and C4
when C1 cleaves C2 and C4 what forms
C2b4b convertase
C2b4b role
coverts C3 to C3b
__ is synthesized in the liver in response to IL-6
c reactive protein (acute phase reactant)
c reative protein activates
classical pathway via C1 binding
C3a role
anaphylatoxin (histamine release mast cells, increased vascular permeability)
stable C3b leads to formation of
MAC
MAC is formed from
C5-C9
C5a role
anaphylatoxin, neutrophil chemotaxis
membrane proteins that protect human cells
decay accelerating factor (DAF/CD55)
MAC inhibitory protein (CD59)
DAF disrupts
C3b attachment
CD59 disrupts
MAC
CD55 and CD59 are especially imp for protecting
RBCs
def of DAF or CD59 leads to
hemolysis
paroxysmal nocturnal hemoglobinuria sxs
sudden hemolysis at night
anemia
abd pain
thrombosis
paroxysmal nocturnal hemoglobinuria due to defect in
DAF/CD59
inherited C3 def sxs
recurrent infections with encapsulated bacteria
immune complex deposition
C5-C9 def sxs
impaired defense against encapsulated bugs (neisseria)
hereditary angioedema due to def in
C1 inhibitor protein
C1 breaks down
bradykinin (vasodilator)
hereditary angioedema diagnosis
low C4 (due to lack of C1 inhibitor)
never give __ to patients with hereditary angioedema
ACE inhibitor (leads to more build up bradykinin)
C3 nephritic factor stabilizes
C3 convertase
CH50 tests
classical pathway (need all compliment factors for normal result)