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32 Cards in this Set

  • Front
  • Back
common pathway
c3->c5->c6->c7->c8->c9
MAC = 5-9
classical pathway
abag
c1q/2r/2s -> c1 -> c14b -> c14b2a
mblectin pathway
MBL-MASP1-MASP2 -> MC4b -> MC42a
alternative pathway
iC3 + Factor B -> (Factor D) -> iC3Bb
rapidly degraded unless properdin
--> C3bBbP [C5 convertase]
c3a,c4a,c5a
anaphylatoxin
which ab can complement
igg not igg4 and igm
c1inh
binds c1r and c1s and blocks c1 complex
factor I
cleaves c4b
rapid inactivation of c3b
factor H
factor I cofactor (c3b)
C4-BP
factor I cofactor
MCP (CD46)
cofactor for factor I
c3b inactivation
DAF (CD55)
dissociation of c3/c5 convertases
S protein (vitronectin)
inhibits MAC insertion (c5b-7)
clusterin
inhibits MAC insertion (c5b-7)
CD59 (protectin)
inhibits MAC formation (c8-c9)
HRF
inhibits MAC formation (c8-c9)
cr1
dissociation of C3/c5, cofactor for c3b/c4b for factor I
factor J
inhibits c1 complex and Bb-mediated cleavage of c3.
cr1
c3b and c4b
cofactor for factor I cleavage of these
alternative pathway deficiencies
neisseria meningitis
factors B + D = AR
properdin = XLR
classical pathway deficiencies
c1, c2, c4
AR
glomerulonephritis & SLE
MAC deficiencies
AR
c5-c9
neisserial infections
def that cause PNH
DAF
CD59
HRF
PIG-A defect
acquired, not inherited.
hereditary angioedema
c1-inh deficiency
(AD)
c4BP deficiency
angioedema
behcet's like syndrome
AR
factor I/H deficiency
pyogenic infections
AR
MBL deficiency
***AD***
URTI's.
ch50 assay
classical pathway measurement
sheep rbc + commercial antibodies + patient serum
activity = reciprocal of dilution that lyses 50%
low ch50 means...(normal ah50)
low c1q/r/s, c2, c4
ah50 assay
rabbit rbc without ab
+ Mg/EGTA
absorb serum with cold rabbit rbc 1st to remove nonspec
low ah50 means...(normal ch50)
low properdin (common)
low factor B or D
low ah50 + ch50 means
low c3/c5/c6/c7/c8/c9