April 4th (Exam 3) Flashcards
What are the primary immunodeficiency diseases caused by?
These are caused by defects in genes that are involved in the immune system.
What are the types of primary immunodeficiency diseases?
Dominant - just one allele needed
Recessive - both alleles needed
What does it mean for a defect to be X linked?
Who is typically affected?
It means that gene is carried on the X chromosome
Males are, females are usually carriers.
Why are most primary immunodeficiency diseases recessive and the ones that are dominant are not severe?
Because the dominant traits likely got selected out when people got them and died.
IFN-gamma is a cytokine that…
And is made by?
Activates macrophages
Made by T (h) 1 CD4 T cells and CD8 T-cells in adaptive response - also by Nk cells in the innate response.
What type of mutations cause the IFN-gamma receptor deficiency?
Which is more severe?
Both dominant and recessive
recessive is worse
What is XLA?
Who gets affected more males or females?
This is the syndrome that happens when there is a defect in BTK kinase thing that is required for development/ differentiation of pre-B cells.
Females.
What is the term for when the bronchi of the lungs get chronically inflamed?
Bronchiectasis
What is HAE?
What is it caused by?
What complement pathway becomes dominant?
this is an autosomal dominant disease that results in a defect in the complement system.
It is caused by by a defect in C1INH which is a C1 inhibitor, so C1r and C1s are permanently disabled.
The classical pathway and the blood clotting pathway become dominant.
What is the phagocytic defect that we talked about?
What is it caused by?
How?
What is the result?
Leukocyte adhesion deficiency
It is caused by a defect in the CD18 gene which is required for neutrophil and monocyte entry into areas of infection.
Cells can’t clear the infection and phagocytes can’t eat bacteria that are opsinized with complement.
What does the defect in IL-12 receptor do?
What is IL-12 used for?
In both the innate and the adaptive immune system, IL-12 is used to stimulate the release of IFN-gamma, but when there is a defect in the receptor we can’t activate macrophages in the innate (NK) and the adaptive immune response (persistent infections)
What are the two strains of the HIV virus?
HIV-1 predominant comes from chimps
HIV-2 less virulent slower progression endemic to west africa and asia now and is from sooty mangabey
What type of virus is HIV? (a subgroup of retroviruses)
What does it contain?
Lentivirus.
- nucleocapsid
- membrane from host
- virus encoded envelope proteins
How do we get the provirus form of HIV?
Integrase integrates the complementary DNA into the host DNA
HIV is an example of an what type of virus? (not endogenous but…?)
exogenous
What are the co-receptors that HIV uses?
CCR5 and CD4 on T, DCs and Macrophages
CXCR4 on CD4 T cells
What is seroconversion?
When abs are detectable.
What do the elite viremic controllers do?
They suppress it
What do they elite neutralizers do?
They create broadly neutralizing abs against it
What are quisi-species?
These are variants of the virus that exist within the infected person.
What is the therapy that uses several antiviral drugs at the same time?
Combinational therapy
What is HAART
