immunodeficiencies Flashcards
test: susceptibility of asplenia:
encapsulated extracellular bacteria
test: susceptibility of c3 deficiency:
recurrent infection with gram-neg bacteria
test: susceptibility of cgd
chronic bacterial and fungal infections, impaired neutrophil response
innate deficiencies to know
factor I and factor H
BTK affected gene? Immune defect? Susceptibility?
BtK, B cells blocked at pro-B-cell stage, recurrent bacterial infection
SCID affected gene? immune defect? Susceptibility?
RAG1 or RAG2, no gene rearrangements in B cells and T cells, all types of infections (no rag1 or 2, no t cells, alot of infections)
tap1 or tap2 enable:
you to get peptides in the ER so you can present whats in the cytosol. Mutation causes Bare Lymphocyte syndrome
mutation in lambda 5?
no b cells produced
Xlinked agammaglobonemia
BTK b cells blocked at pro-B cell stage, results in recurrent bacterial infections
aire?
transcription factor that drives the expression of non-thymic proteins in the thymus so you can get a broader negative selection
ipex
mut is in foxp3, makes you a regulatory tcell bc you ALWAYS need foxp3
whats the danger in repeated t cell stimulation?
start recognizing self too well
initial hiv infection includes
making antibodies, spike in cd8 t cell activity, the infections virus lays low in plasma until t cells deplete and begin to drop
AFT works to:
rapidly lowers viral load in hiv patients
corticosteroids TEST:
inhibit inflammation, inhibit many targets including cytokine production by macrophages, nuclear option
azathioprine, (cyclophosphamide) mycophenolate TEST!!!! more than the others
(methotrexate)
inhibit proliferation of lymphocytes by :interfering with DNA synthesis!!! that’s why they’re also used as chemotherapy drugs
Cyclosporin A tacrolimu (fk506) TEST
inhibit the calcineurin-dependent activation of NFAT, block il-2 production and proliferation by t cells
rapamycin (sirolimus) TEST!!
inhibits proliferation of effector T cells by blocking rictor-dependent mTOR activation, and can’t signal through mTOR, you can’t grow, almost all cells need mTOR for growth, to grow, need more proteins, no mTOR
lymphocytes and organ rejection, they’re all:
trying to prevent expansion, preventing activation, preventing killing new organ; block Ca signal, growth pathways rapamycin (mTOR) or DNA synthesis azathioprine, (cyclophosphamide) mycophenolate
biologics
have well defined targets, bc we know variable region, constant regions, what pathways they should efect rituximab
rituximab
specified against anti-CD20, eliminates B cell
natalizumab!! TEST
anti-alpha4 integrin, block lymphocyte buffering, MS
omalizumab TEST
anti-IgE removes IgE antibody, chronic asthma
Ipillmumab TEST
Anti-CTLA-4, Increases CD4 Tcell responses, metastatic melanoma
