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