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Complement

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zijimubo1's version from 2016-12-14 09:45

Section

Question Answer
C1 esterase inhibitor (C1-INH)inhibits C1r and C1s; leads to hereditary angioedema; can be test by LOW C2 and C4 levels
RCA proteins (Regulatory Complement Activation)regulate C3 and C5 convertase; microbes lack RCA proteins
Vitronectin (S protein)regulates MAC by binding/blockin C5b-7
Clusterin (SP-40)regulates MAC by binding to C5b-C9
CD59binds C9 found in RBCs WBCs neuronal and endothelial cells. Anchored by GPI
RCA protein with co-factorsCD46; Factor I; cleaves C3 and C4
RCA proteins with Decay-accelerationCD55; disrupts association of C3 with C2a or Bb
Factor H and I defectsuncontrolled activation - glomerullonephritis; Atypical hemolytic uremic syndrome
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HLA

Question Answer
8-10 peptidesHLA I
12-30 peptidesHLA II
HLA II genesDP, DQ, DR (both with B A) on chromosome 6
HLA I genesB C A and B2M on chromosome 15
MHC "restricted" T cellself-MHC+ peptide
Non restrictednon self-MHC or wrong peptide
T-cells are restricted to respond only to what?self MHC molecules
Peptides are restricted to bind to what?self-MHC molecules
Class I binds peptides fromintracellular compartment
Class I binds peptides fromextracellular compartment
HLA I molecules present on surface throughClassical and Cross Presentation pathways
Classical presentation pathwayintracellular virus and bacterial break through proteasome, enter ER via TAP, loaded to MHC on ER, transported to golgi to cell surface
Function of ERAPto cleave the antigen down to 8-10 peptides to be able to present on MHC
Cross presentation (extracellular) pathwayengulfed, retrotransport followed by classical (Proteasome and TAP)
What is special about cross presentation pathwayAPCs only, Class 1, and its extracellular, Retrograde transport
What is retrograde transport?engulfed vesicle releases substance instead of fusing with lysosome
Classical pathway for MHC IIextracellular(exogenous) ; degradation by CAPETHSINS, forms MIIC endosome; meanwhile you have translation of MHC II in the ER with invariant chain(CD74), released with CLIP. Fusion with MIIC, and HLA-DM removes clip allowing peptide to bind. Then surface expression.
Class I-like CD1 Moleculelooks like an MHC but contains a glycolipid instead allowing for expression of different types of antigens
What are Superantigens?Binds to Class II, thus activating CD4 releasing a lot of cytokines. Activate outside of groove.
BLS-1 defectdefect in HLA class 1 usually TAP
BLS-2 defectdefect in HLA class 2 due to changes in transcription. CIITA transcription factor
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