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16 Cards in this Set
- Front
- Back
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Chronic granulomatous disease
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F(x)al deficiency:
-Defective ROS production (phagocytes) Mechanism: -Mxn: phagocyte (NADPH) oxidase ez (usu Cytochrome b588) |
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Leukocyte adhesion deficiency - 1
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F(x)al deficiency:
-X/def B2-integrin expression -> defective LADep functions Mechanism: -Mxn: B-chain (CD18) of B2 integrins |
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Leukocyte adhesion deficiency - 2
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F(x)al deficiency:
-X/def leukocyte ligand expression for E/P-selectin -> no leukocyte migration into tissues Mechanism: Mxn: ptn for E/P-selection ligand synthesis |
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Complement deficiency (C3)
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F(x)al deficiency:
-Def complement cascade activation Mechanism: -Mxn: C3 |
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Complement deficiency (C2, C4)
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F(x)al deficiency:
-Def activation of classical complement -> failure to clear immune complexes + dev lupus-like disease Mechanism: -Mxn: C2 or C4 |
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Chediak-Higashi syndrome
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F(x)al deficiency:
-Def lysosomal function -Neutrophils/m-phages/DC's -Def NK granule f(x) Mechanism: -Mxn: lysosomal trafficking ptn (TAP??) |
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SCID:
X-linked SCID |
F(x)al deficiencies:
-\/ T -Nml / /\ B -\/ serum Ig Mechanism: -Mxn: (cytokine receptor) common-gamma-chain - -> def T maturation d.t. lack of IL-7 |
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SCID:
AR SCID (d.t. ADA/PNP deficiencies) |
F(x)al deficiencies:
-Prog. dec in T/B (mostly T) -ADA def: Dec serum Ig PNP def: Nml B/Ig Mechanism: -Toxic metabolites accumulate in lymphocytes |
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SCID:
AR SCID (d.t. other causes) |
F(x)al deficiencies:
-Dec T and B -Dec serum Ig Mechanism: -Def T/B maturation -Mxn: POSSIBLY RAG genes |
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B cell ID's:
X-linked agammaglobulinemia |
F(x)al deficiencies:
-Dec all serum Ig isotypes -Dec B Mechanism: -Mxn: Btk -No B maturation past pre-B |
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B cell ID's:
Ig heavy-chain deletions |
F(x)al deficiencies:
-IgG1, IgG2, or IgG4 absent -Sometimes IgA / IgE absent Mechanism: -Deletion @ 14q32 (Ig HC locus) |
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T cell ID's:
DiGeorge syndrome |
F(x)al deficiencies:
-Dec T -Nml B -Nml / dec serum IG Mechanism: -Thymic hypo-/aplasia -D.t.: Aberrant 3rd/4th branchial pouch formation |
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X-linked hyper-IgM syndrome
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F(x)al deficiencies:
-Def in activation of helper T-dependent B cell, and macrophage Mechanism: -Mxn: CD40L |
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Common variable immunodeficiency (CVID)
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F(x)al deficiencies:
-Dec/no production of selective Ig isotypes/subtypes -Nothing OR bact infxn susceptibility Mechanism: -Mxn: B cell GF receptor, costimulators |
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Defective class I MHC expression:
"Bare lymphocyte syndrome" |
F(x)al deficiencies:
-No class II MHC expression -> impaired CD4+ activation -Def cell-mediated + T-dependent humoral immunity Mechanism: -Mxn: TF's for MHC II expression |
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Defects in TCR complex expression / signaling
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F(x)al activity:
-Dec T, or abnormal ratios of CD4/CD8 subsets -Dec cell-mediated immunity Mechanism: -Mxn/deletion: CD3, ZAP-70 |
