Chapter 13

Question 1

Diseases are caused by inherited gene defects (congenital)
-adaptive usually but worse when it’s in innate

Answer 1

Primary immunodeficiency

Question 2

Are acquired as a consequence of other diseases or result from other issues such as starvation or medical intervention

Answer 2

Secondary immunodeficiency

Question 3

Primary immunodeficiency diseases are caused by inherited gene defects in ____

Answer 3

Cell development

Question 4

Most gene defects resulting in immunodeficiency are ___and many caused by mutations in __chromosome

Answer 4

Recessive; X

Question 5

-problem with cytokine signaling
-men are more likely to have
-women are carriers but must be on active X

Answer 5

Severe combined immunodeficiency (SCID)

Question 6

SCID is caused by mutations in gene ____ on X. It encodes _____receptor. Mutation causes problem in signaling of?

Answer 6

1. IL2RG
2. IL2
3. All IL2 family cytokines. IL2 family forces cells to differentiate into Treg

Question 7

If _____ and _____don’t work T cells and NK don’t properly develop

Answer 7

IL7 and IL15 (needed for survival of CD4 and CD8 memory T cell; maintenance of memory)

Question 8

Specific abnormality: Thymic aplasia
Immune defect: variable numbers of T cells
Susceptibility: general

Answer 8

Digeorge syndrome

Question 9

Specific abnormality: mutations in TAP 1, TAP2 and tapsin
Immune defect: no CD8 T cells
Susceptibility: chronic lung and skin inflammation

Answer 9

MHC class I deficiency

Question 10

Specific abnormality: lack of expression of MHC class II
Immune defect: no CD4 T cells
Susceptibility: general

Answer 10

MHC class II deficiency

Question 11

What can the defects in immunodeficiency diseases be?

Answer 11

1. Defects in T cell development
2. Defects in signaling
3. Defects in B cell development result in deficiency’s in antibody production that cause an inability to clear extra cellular bacteria and some viruses

Question 12

B cell defects can lead to problems in ____

Answer 12

Class switching

Question 13

-born with high level of maternal ___ (moves across placenta)
-after birth, ___ starts immediately

Answer 13

IgG; IgM

Question 14

IgG starts at ___ months; total IgG falls because maternal IgG catabolized
- IgG levels are low from ____ to ____

Answer 14

6 months; 3 months to 1 year of infant

Question 15

In males stimulation of the pre B-cell receptor recruits cytoplasmic proteins including ____ to transduce a signal that triggers B cell development.

Answer 15

BTK (Brutons tyrosine Kinase)

Question 16

In males the X-linked agammaglobulinema (XLA) the ____ gene on X chromosome is defective

Answer 16

BTK protein

Question 17

-tyrosine protein
-IgM will only have BTK signal transduction

Answer 17

BTK protein

Question 18

In male with XLA ___ signal transduced even though receptor is there

Answer 18

no

Question 19

In females half of the pre-B cells will have be expressing the defective BTK gene and will not develop further. This is since one of the two X chromosomes in each cell is ______ early in development

Answer 19

permanently inctivated

Question 20

Defects in activationand differention will have an impact on cell mediated and ____ response

Answer 20

antibody

Question 21

defects specific to activation and differentiation of B cells can impair their ability to do _____ and leave cell mediated response intact

Answer 21

class switching IgG, A, or E

Question 22

Common feature of patients with defects in B cell class switching is ____. have normal B and T cell development and normal or high serum IgM, but make limited antibody response that require T cell help

Answer 22

hyper-IgM syndrome

Question 23

In this syndrome you can not class switch, have build up of M and long term immunity is effected because you can not make IgG

Answer 23

hyper-IgM syndrome

Question 24

In X-linked hyper IgM syndrome: mutation in ______. This is on activated T cells allowing them to engage with CD40 antigen presenting cells.
-patients have severely reduced circulating antibody levels of all except IgM and are highly susceptible to bacterial infections

Answer 24

CD40 Ligand

Question 25

In males with hyper-IgM syndrome in the CD 40 Ligand the B cells are normal, But ________.

Answer 25

dont engage with T cells, dont undergo isotype switching or intiate the formation of germinal centers

Question 26

Can have inherited defects in signaling pathways CD4 T cells -Normal pathways for host defense against different infectious agents are pinpointed by genetic deficiencies of cytokine pathways central to _____ and ___ responses -defects are not in antibody production, but in the ____ pathways. -such defects make them susceptible to some bacterial infections

Answer 26

1. Type 1/TH1 and Type 3/TH17 2. signaling

Question 27

can have inherited defects in signaling pathways (CD8 T cells) -inherited defects in the cytolytic pathway of lymphocytes can cause uncontrolled _____ and ____ responses to viral infections

Answer 27

lymphoproliferation and inflammatory responses

Question 28

while there is impaired release of sytotoxic granules, there is uncontrolled activation and expansion of CD 8 T cells and macrophages that infilitrate multiple organs, cause tissue necrosis and organ failure. _____ is released from ___ and ___ and this leads to ______

Answer 28

1. IFN-gamma (which brings macrophages and inflammatory cytokines) 2. CTLs 3. NK 4. greater activity of macrophages, and pro-inflammatory cytokines such as TNF, IL-6 and CSF

Question 29

Defects in complement components and complement regulatory proteins cause ____ and tissue daamage

Answer 29

defective humoral immune function

Question 30

In classical pathway (C1, C2 and C4) deficiency leads to ____. Antibody and antigen combine but nothing else happens.

Answer 30

immune complex diseases

Question 31

In MBL pathway (MBL, MASP1, MASP2, C2, C4) deficiency of MBL leads to ____, mainly in childhood

Answer 31

bacterial infections

Question 32

In the alternative pathway (Factor D and Factor P (regulate complement)) deficiency leads to infection with pyogenic bacteria and Neisseria spp. but ____

Answer 32

no immune complex disease (more genetic problems in the human being)

Question 33

___ deficiency leads to infections with pyogenic bacteria and Neisseria spp. sometimes immune complex disease

Answer 33

C3

Question 34

membrane attack components (C5, C6, C7, C8, C9) deficiency leads to infection with ____ only

Answer 34

Neisseria spp.

Question 35

_____ or gene therapy can be useful to correct genetic defects

Answer 35

hematopoietic stem cell transplantation

Question 36

-4 different things bacteria/ virus can do to evade the immune system 1. extracellular bacterial pathogens have evolved different strategies to avoid detection by ____ and destruction by antibody, complement and antimicrobial peptides 2. Intracellular bacterial pathogens can invade the immune system by _____ 3. RNA viruses use different mechanisms of ____ to keep a step ahead of the adaptive immune system 4. DNA viruses use multiple mechanisms to subvert ___ and ___ responses

Answer 36

1. pattern recognition receptors 2. seeking shelter within phagocytes 3. antigenic variation 4. NK cell and CTL

Question 37

what are the 5 ways extracellular bacteria avoids detection?

Answer 37

sheilding on MAMPs, antigenic variation, inhibition of oposonization, inhibition of ROS and resistance to antimicrobial peptides

Question 38

change the antigen so it not recognized like spike porteins

Answer 38

antigenic variation

Question 39

bacteria have capsule so makes it difficult for C3b to land for oponization

Answer 39

Inhibition of opsonization

Question 40

Intracellular bacteria evade the immune system .... ____(inside macrophages) -tuberculosis infects lungs and manifests by coughing -causative agent of leprosy

Answer 40

mycobacterium

Question 41

Intracellular bacteria evade the immune system .... ____(spinach) escape from phagosome into the cytoplasm of macrophage where they multiply

Answer 41

listeria monocytogenes

Question 42

Intracellular bacteria evade the immune system .... ____uses type III secretion system to secrete SifA into host cytosol and membranes to alter composition of ___ containing vacuole to avoid destruction

Answer 42

Salmonella

Question 43

In salmonella 1. Gram negative organisms have type III or type Iv secretion systems or injectisomes 2. assembly of proteins on membrane 3. bacterial proteins injected into host cell 4. these virulence factors aid in escaping inmmune response: block signaling cascade ____and ____

Answer 43

NFkB and MAP kinases

Question 44

_____ (causative agent of plaque) YOP proteins which inject into phagocytes, destroys the actin cytoskeleton, which is needed for phagocytosis

Answer 44

Yersinia pestis

Question 45

_____destroy cytoskeleton through inject system and secretion system. when the cytoskeleton is destroyed it is called Ruffle. it allows microorganisms to enter.

Answer 45

salmonella

Question 46

1. several pattern recognition receptors that recognize microbial forms of DNA and RNA produced in the course of viral infection and replication activate the production of ___ by the infected cell 2. heterodimeric receptors for these cytokines are expressed on nearly all cells of the body and activate ____ and ____ to amplify production of the interferons and induce expression of restriction factors that block replication of viruses 3. Key sites in the interferon signaling cascades are blocked by many viruses

Answer 46

1. interferons 2. STAT 1 and STAT 2

Question 47

antigeneic changes from reassortment of segments of RNA genome leads to ____= global pandemics every 10-50 years

Answer 47

antigenic shift

Question 48

-overtime antigens change, for example influenza it changes all the time, the surface proteins are changing -neutralizing antibodies against hemagglutinin block binding to cells -mutations alter epitopes in hemagglutinin so that neutralizing antibody no longer binds

Answer 48

antigenic drift

Question 49

-occurs when RNA segments are exchanged between viral strains in a secondary host -no cross protective immunity to virus expressing a novel hemagglutinin

Answer 49

antigenic shift

Question 50

DNA viruses have.... 1. _____ but employ antigenic variation. Have larger genomes, more proteins that help them fight off immune cells. (herpes viruses: latency, no viral proteins loaded on MHC I) 2. ______ (viral homologs of MHC I which ends up binding NK cells and blocks its action) 3. Viral homologs of cytokines and their receptors. Decoy receptors that interfere with ___ or ______

Answer 50

1. low mutation rate 2. repress cytolytic activity of NK cells 3. natural ligand signaling, or make proteins that inhibit JAK/STAT signaling

Question 51

many proteins which prevent the appearance of viral peptide : MHC I on the infected cell

Answer 51

immunoevasins

Question 52

some viral proteins degrade the newly made MHC I molecule using a pathway that usually degrades misfolded proteins

Answer 52

dislocation

Question 53

Latency of viruses 1. these viruses enter latency by expression of small region of their genome called ____ 2. This suppresses the transcription of viral genome, produces factors that interfere with apoptotic host cell death, immune mechanisms that could clear death -herpes goes latent and hides from immune system, hiding in the trigeminal ganglion. it hides in nerves because it is non dividing and it comes out in stress. herpes produces products to keep the ganglion alive and prevents the immune system from reaching the ganglion.

Answer 53

LATs (latency associated trancript)

Question 54

____ replicates to higher viral loads in blood, high rate of transmission from mother to child -it progresses more rapidly -originated from SIV four sperate transmissions to humans from chimpanzees or low land gorillas -went through antigenic drift so now it can infect humans, it switched species

Answer 54

HIV-1

Question 55

___originated in the sooty mangabey monkeys

Answer 55

HIV-2

Question 56

HIV-1 is complicated to make a vaccine because of all the different types of ___

Answer 56

clades

Question 57

means that is slow progression

Answer 57

lentivirus

Question 58

- ____is a retrovirus that establishes a chronic infection that slowly progresses to AIDS -HIV infects and replicate within cells of the immune system -activated CD4 T cells are the major source of HIV replication - there are several routes by which HIV is transmitted and establishes infection -HIV variants with tropism for different co-receptors play different roles in transmission and progression of disease

Answer 58

HIV

Question 59

what is HIV looking for?

Answer 59

T cells and macrophages. CD4 t cells looking for CCR5 receptor

Question 60

___target effector memory (TEM) and resident memory (TRM) CD4 T cells, dendritice cells and macrophages. ___ of HIV are thought to represent the major population of virus that establishes initial infection. -wants CCR5

Answer 60

R5 variants

Question 61

HIV virions that bind the ___ chemokine receptor so called X4 variants) target naive and central memory (TCM) CD4 T cells. X4 variants of HIV emerge after initial infection

Answer 61

CXCR4