Immuno Lec 28.txt
Card Set Information
Immuno Lec 28.txt
Immuno 28 immunodeficiencies
X-linked hyper IgM syndrome?
Can't produce Plasma cells or macrophages
CD affected by X-linked hyper IgM syndrome?
AID mutations, autosomal hyper IgM syndrome?
Can't mature B-cells to Plasma cells
Macrophages do not produce RO species = fibrous tissue laid down with T-cells and fibrocartilage
Beta-2 integrins shot so binding does not occur
What CD is affected in LAD1?
Sialyl-Lewis X component of E/P slectin ligands causes inability of lymphocytes to migrate
Deficient complement cascade
Complement C2, C4 deficiency?
Can't activate classical pathway
no clearing of pathogens
Defective LYST gene
Neutrophils, marophages, dendrites and NK cells do not function correctly
impaird binding of proteins and vesicles as well as granules
can't produce immature B-cells
Lack of thymus
ineffective T-cell maturation
inefficient gamma-chain on cytokine receptors
What cytokines are affected by Deficient CD132?
T, NK and B cells for CD 132?
T/NK cells are not present
B-cells are non-functional
Adenosine deaminase (ADA)?
can't produce purines
buildup of toxic metabolites and inhibition of DNA synthesis
No JAK-STAT pathway
no transcription factors for gene regulation
What cells are damaged b/c of JAK3 deficiency?
B and T-cells
Recombination activating gene (RAG) deficiency?
inhibit T and B cell development via lack of TCR and Ig's
NK cells not affected
Wiskott-Aldrich syndrome (WAS)?
ineffective WASP protein
What cells are affected by WAS?
symptoms of WAS?
Normal/Increased IgA, IgG, IgE
Mutation in ATM kinase
lack of DNA repair
Symptoms of AT?
dilated blood vessels
Antibodies affected by AT?
IgA, IgE, IgG all decreased
Hyper IgE syndrome?
Lack of STAT3 and lack of IL-12 production
Symptoms of Hyper IgE syndrome?
Lack of phagocytosis
Th2 overproduction = high IgE production
pneumonia, abscesses, facial, skeletal, and dental abnormalities