3.1 Flashcards

1
Q

Immunodeficiency diseases

A

Inherited diseases: Pathogens evade and subvert the immune system

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2
Q

Type of Immunodeficiency disease: Streptococcus pneumoniae (90 Serotypes)

A

Numerous different strains
Antigen variability
Long-term immunity decreased

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3
Q

Antigenic Drift

A

Process by which point mutations in influenza virus genes causes alterations in the structure of viral surface antigens causes year-to-year antigenic variation

Common in influenza and avian viruses

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4
Q

Mutation and recombination

A

Generation of new viral strains that avoid immunity

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5
Q

RNA viruses more prone to errors

A

Influenza, HIV and Covid

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6
Q

Antigenic Shift

A

Process by which influenza viruses reassort their segmented genomes and change their surface antigens radically - shifts are usually the cause of influenza pandemics

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7
Q

Epidemic versus Pandemic

A

Mutation and antigenic drift (epidemic) versus recombination and antigenic shift (pandemic)

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8
Q

Trypanosoma brucei

A

protozoan
Africa - cattle and humans

Insect and animal host - genome encodes for 1000 genes encoding for variable surface glycoproteins (VSGs)

Gene conversion - gene in the expression site is excised and replaced by a copy of a different homologous gene

Host makes defense to dominant VSG form not the new minority VSG selection for minority

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9
Q

Salmonella typhimurium and Neisseria gonorrhoeae

A

Dramatic cycling in the number of parasites in the individual - causes inflammation and neuro damage
leads to coma

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10
Q

Viruses cleared by

A

cytotoxic CD8 T-cells

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11
Q

HSV

A

latent
Infects epithelial cells then sensory neurons
cleared from epithelial cells but latent in neurons
Reactivation and it travels down the sensory neuron (trigeminal) to the epithelium

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12
Q

why are neurons a good site for latent viruses?

A
express small numbers of MHC class I molecules 
do not recognize they are infected
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13
Q

Latent viruses in neurons

A

Herpesvirus varicella-zoster (chicken pox/shingles) and Epstein-Barr Virus (EBV)

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14
Q

Mycobacterium tuberculosis

A

Prevents the fusion of phagosome with lysosome in macrophages - flourishes in the vesicles

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15
Q

Toxoplasma gondii

A

encloses itself in an impenetrable membrane-enclosed vesicle that does not fuse with other vesicles - prevents antigen processing

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16
Q

Treponema pallidum (syphilis)

A

coats itself with human proteins to evade antibody binding

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17
Q

Virus info

A

Greatest ability to evade human immune system
replication and life cycle depend on metabolic and biosynthetic processes of human cells
Capture of cellular genes encoding for cytokines or their receptors
synthesis of proteins that prevent complement fixation or antibody processing and presentation to CD8 T cells
Need host to survive and replicate

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18
Q

Cytomegalovirus (CMV)

A

Latent
10 proteins to diminish MHC I from stimulating NK cells and CD8 T cells
158 million people infected - few initial symptoms remains latent
life-threatening to immunocompromised people

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19
Q

Bacterial Superantigen

A

Small bacterial protein - toxin
Can crosslink MHC II on APC with TCR on CD4 T cell
Non-specific activation of CD4 T cells (2-20%) and excessive production of Il-2 INF-y and TNF-a confuse innate immune response
Lots of resources for a less specific response

20
Q

What uses bacteria superantigens to confuse the body?

A

Streptococcus aureus and streptococcus pyogenes

21
Q

S. Aureus

A

Staphylococcal superantigen-like proteins (SSLPs)
SSLP7 - prevents monomeric IgA from delivering bacteria to phagocytes
Contains binding sites for Fc region of IgA and C5 complement

22
Q

Primary immunodeficiency disease

A

Inherited defects

enhanced susceptibility to infection or autoimmunity

23
Q

Secondary immunodeficiency disease

A

Due to environmental factors (Immunosuppressive drugs)

24
Q

IFN-y

A

Dimer complex
only wild-wild signals
activates macs
Made by NK cells (innate) and Th1 CD4 and CD8 t cells (adaptive)

25
Q

Antibody deficiency

A

Poor clearing of extracellular bacteria

26
Q

Encapsulated bacteria

A

S. aureus
S. pneumoniae
P. aeruginosa
- often evade innate immunity

27
Q

X-linked agammaglobulinemia (XLA) - Bruton’s tyrosine kinase (BTK) defect

A

required for B-cell activation and pre-B cell development and differentiation
Antibiotic treatable - successive treatments can lead to tissue damage (excessive release of proteases from bacteria and phagocytes) - airways- bronchiectasis

There is an absense of mature B cels and all immunoglobulins
Recessive x link pattern (on x)

Treatable with monthly injections of immunoglobulin - antibodies against common pathogens (passive immunity)

28
Q

Autosomal Agammaglobulinemia’s (non-Bruton type)

A

Can be caused by mutations in a large number of genes

Usually, autosomal recessive but there have been dominant cases in families

29
Q

Phagocytosis defects

A

enhanced susceptibility to bacterial infection

30
Q

Leukocyte adhesion deficiency (LAD-1)

A

Autosomal recessive
Phagocytic disfunction
Defect in the LFA-1 integrin proteins on phagocytes which prevents their adherence and migration out of the blood vessels

Presents with recurrent bacterial infections, absent pus formation, poor wound healing and delayed separation of the umbilical cord
Neutrophilia will be observed on a blood smear

31
Q

Chediak-Higashi Syndrome (CHS)

A

Characterized by recurrent staph/strep infections, partial albinism, and peripheral neuropathy (nystagmus)
Caused by defect in LYST (lysosomal trafficking regulator) gene which leads to microtubule dysfunction in phagocytes
Autosomal recessive

3 Ps
Pyogenic infections (strep/staph)
Partial albinism
Peripheral neuropathy

Giant granules in neutrophils on blood smear

32
Q

Chronic Granulomatous disease (CGD)

A

Deficiency of neutrophil NADPH oxidase - cannot make hydrogen peroxide
Neutrophils are dysfunctional
susceptibility to infection by catalase-producing organisms
highly susceptible to bacteria that break down hydrogen peroxide (catalase-positive organisms)
ex: S. Aureus; E. coli; Psudomonas; and Aspergillus

treatment is lifelong regimens of antibiotics and antifungals to prevent infections
interferon-gamma treatment and
hematopoietic stem cell transplant

33
Q

C1-C4

A

Classical pathway is required for elimination of immune complexes - CR1 binds C4b and C3b attached to the complex and transports to RBCs.
Accumulation of immune complexes in blood and deposition of immune complexes in tissue
activates phagocytes increasing inflammation and tissue damage

34
Q

C3

A

Opsonin that promotes the efficient elimination of bacteria by phagocytes
Susceptibility to encapsulated bacteria

35
Q

C4-C9

A

Terminal complement components of the membrane-attack complex
increased susceptibility to Neisseria infections

36
Q

Hereditary Angioedema

A

Deficiency in the C1 esterase inhibitor (C1NH)
Autosomal dominat
Eyelids, lips, genitals, hands and feet
Localized edema, puffy skin and swelling

Treatment involves Danazol (a synthetic androgen and partial agonist at the androgen receptor; promotes C4 synthesis and increase in C1-NH
replacement therapy with C1NH

37
Q

T cell dependent antibody response and cell-mediated immune response deficiency

A

SCIF - severe combined immune deficiency

38
Q

SCID (severe combined immunodeficiency)

A

X-linked defect leading to an impaired common gamma chain on cytokine receptors
- mainly an absence of t cells

or cellular absence of adenosine deaminase (ADA) or purine nucleoside phosphorylase (PNP) - auto recessive
- absence of B, t and NK cells

39
Q

DiGeorge Syndrome

A

Spontaneous deletion of part of chromosome 22
No proper formation of the thymus - decreased/ absent t-cell mediated immune response

treatments
antibiotics 
calcium supplements 
ear tubes or hearing aids
occupational therapy 
physical therapy 
replacement of missing hormones 
surgery
40
Q

Wiskott-aldrich syndrome

A

x-linked recessive
presents early in childhood in males with recurrent bacterial infections, eczema, purpura, epistaxis (nosebleed) and splenomegaly
IgM low
IgE and IgA are high
WASp is the mutated x gene
- WASp is expressed only in hematopoietic cells

41
Q

Hyper-IgM Syndrome

A

Absence of other immunoglobulins
Deficiency in CD40 Ligand
require immunoglobulin replacement therapy

42
Q

AIDS

A

Massive reduction in CD4 t cells and severe infection from pathogens that rarely cause trouble in healthy individuals (opportunistic infection)

43
Q

HIV 1

A

Chimps

44
Q

HIV 2

A

Monkey

45
Q

HIV

A

Exogenous retrovirus

spike protein

46
Q

CCR5 deficiency

A

White population - 10% heterozygous 1% homo

deletion mutant resistant to HIV infection

47
Q

HAART

A

highly active anti-retroviral therapy 1997