immunogenetics Flashcards by Morgan C

what does the disufide bond connect on an antibody

2 heavy chains

How well did you know this?

Not at all

Perfectly

what part of the antibody activates complement and phagocytes

constant region

How well did you know this?

Not at all

Perfectly

what part of the antibody binds to antigen

variable region

How well did you know this?

Not at all

Perfectly

MCH is essential for…

antigen presentation to T cells

How well did you know this?

Not at all

Perfectly

self MHC + foreign antigen=

T cell response

How well did you know this?

Not at all

Perfectly

self MHC + self antigen=

no T cell response

How well did you know this?

Not at all

Perfectly

no MCH + foreign antigen=

no t cell response

How well did you know this?

Not at all

Perfectly

where is mhc located

every nucleated cell in the body

How well did you know this?

Not at all

Perfectly

MHC 1 alters what

CD 8 + T cells because viruses infect these

How well did you know this?

Not at all

Perfectly

____ is a key factor for determining tissue matching for transplant donors and recipients

MHC

How well did you know this?

Not at all

Perfectly

MHC molecules have a broad specificity for peptides meaning….

many diff peptides can bind in the MHC binding cleft

How well did you know this?

Not at all

Perfectly

true or false: peptides associated with mhc have a slow on and off rate

true

How well did you know this?

Not at all

Perfectly

mhc haplotype of a person determines what

which peptides bind and how peptides bind

How well did you know this?

Not at all

Perfectly

mhc genes are highly what

polymorphic

How well did you know this?

Not at all

Perfectly

how many mhc alleles are in humans? how many recombinations?

hundreds of alleles with 10^13 recombinations

How well did you know this?

Not at all

Perfectly

why is it hard to find transplant donors

mhc genes are highly polymorphic

How well did you know this?

Not at all

Perfectly

how are mhc genes expressed?

condominantly meaning that all alleles are expressed at equal frequency

How well did you know this?

Not at all

Perfectly

mhc haplotype?

set of mhc alleles on an individual chromosome

How well did you know this?

Not at all

Perfectly

mhc haplotype can influence…

how an individual responds to certain pathogens
susceptibility to certain diseases
transplant success

How well did you know this?

Not at all

Perfectly

first primary immunodeficiency disease to be descibed?

Bruton agammaglobulinemia

How well did you know this?

Not at all

Perfectly

when are primary/congenital immunodeficiencies frequently manifested?

infancy and childhood

How well did you know this?

Not at all

Perfectly

what are secondary/acquired immunodeficiencies a consequence of?

malnutrition, disseminated cancer, immunosuppressive drugs, infection of cells of the immune system

How well did you know this?

Not at all

Perfectly

what is conserved across widely diverse species

TLR

How well did you know this?

Not at all

Perfectly

what do primary immunodeficiency disorders affect

B cells, T cells, NK cells, phagocytic cells, and complement proteins

How well did you know this?

Not at all

Perfectly

what may immunodeficiencies result from

defects in leukocyte maturation/activation or defects in effector mechanisms of innate and adaptive immunity

principle consequence of immunodeficiency?

inc susceptibility to infection

nature of infection in a pt depends on what

component of the immune system thats defective

deficient humoral immunity results in inc susceptibility to fight infection by what

pyogenic bacteria

what antibody isotopes are low in XLA/brutons agammaglobulinemia

ALL

are circulating B cells present in XLA/brutons agammaglobulinemia

are pre B cells present in XLA/brutons agammaglobulinemia

yes but they are reduced in number in the bone marrow

tell me about the tonsil and lymph nodes in XLA/brutons agammaglobulinemia

tonsils are very small and lymph nodes are not palpable

why are tonsils are very small and lymph nodes are not palpable in XLA/brutons agammaglobulinemia

absence of germinal centers

tell me about the thymus in XLA/brutons agammaglobulinemia

theyre normal

who is mainly affected with XLA/brutons agammaglobulinemia

boys

why do boys not usually show symptoms of XLA/brutons agammaglobulinemia during the first 6-9 months of life

they still have the maternally transmitted IgG antibodies

what happens after the maternal IgG antibodies wear off in XLA/brutons agammaglobulinemia

boys repeatedly get infections with extracellular pyogenic organisms such as pneumococci, streptococci, and haemophilus

what type of defect is in XLA/brutons agammaglobulinemia

maturation of B cells

in XLA/brutons agammaglobulinemia, circulation B cells are____ pre B cells are_____

circulation B cells are absent | pre B cells are reduced

what disease is associated with a loss of function of bruton tyrosine kinase

XLA/brutons agammaglobulinemia

what is X linked immunodeficiency with hyper IgM characterized by

low serum IgG, IgA, IgE and increased IgM

when do ppl with X linked immunodeficiency with hyper IgM usually become symptomatic

1st or 2nd year of life

what types of infections do ppl with X linked immunodeficiency with hyper IgM or XLA have

pyogenic infections such as otitis media, sinusitis, pneumonia, tonsillitis

what do pt with X linked immunodeficiency with hyper IgM that pt with XLA not have

hymphoid hyperplasia

what is the defect in in X linked immunodeficiency with hyper IgM

loss of function of CD40 AKA CD154 ligand that's expressed on helper T cells

loss of CD40 or CD 154 prevents what

T cell from co-stimulating antigen specific B cells

in this disease, B cells are not signaled by T cells to go through isotope switching and only make IgM

X linked immunodeficiency with hyper IgM

trmt for humoral immunity deficiency

prophylactic antibiotics and/or gamma-globulin therapy

2 diseases of humoral immunity?

X linked immunodeficiency with hyper IgM and XLA/brutons

deficient cell mediated immunity results in inc susceptibility to fight infection by what

viruses and other intracellular pathogens

trmt for deficient T cell responses?

there arent really any (BMT and gene therapy for now); pts usually only live til infancy or childhood

what does not develop in DiGeorge syndrome

thymus and therefore T cells

what causes thymic hypoplasia in DiGeorge synd

defects in morphogenesis in 3rd and 4th pharyngeal pouches during early embryogenesis

what happens with B and T cells in DiGeorge synd

T cells decreased and B cells increased

how do most infants with DiGeorge synd die in first few months/years of life

infections, CV defects, seizures

where are clinical similarities btwn DiGeorge synd and what other synd

fetal alcohol

what is there a deficiency in in XLR SCID?

T and B cell function; they have few or no T cells and NK cells, but increased B cells

mc form of SCID

X liked SCID/XSCID

what do infants with XSCID present with in 1st few months of life

diarrhea, pneumonia, otitis, sepsis, cutaneous infections

candida albicans, pneumocystis carinii, varicella, measles, parainfluenzae, cytomegalovirus, and EBV are in what disease

XLR SCID

what do pts with XLR SCID lack

ability to reject foreign tissue so theyre at greater risk for GVHD

what can GVHD result from

maternal T cells that cross into the fetal circulation while the SCID infant is in utero

what is wrong with B cells in XLR SCID

they dont produce Ig normally

what are the 2 aims of current trmts of immunodeficienceis

minimize and control infection and replace defective/absent components

trmt of choice for immunodeficiencies rn?

BMT