Abai: Chemotaxis, Immune Mechanisms

Front 1

CC chemokines are expressed on what cell types?

What diseases are associated with CC chemokine defects?

Back 1

Eos, basos, monos

asthma, allergies
(CCL2, 5, 7, 11)

CCL5 = RANTES
CCL11 = Eotaxin

Front 2

CXC chemokines are expressed on what cell types?

Name one diseases that is associated with CXC chemokine defects?

Back 2

neutrophils

CXCR4, WHIM

Front 3

What is the difference between ELR (enzyme link receptors) CXC chemokine receptors?

Back 3

ELR are angiogenic using CXCR2

Non-ELR - angiostatic, CXCR3B and induced with interferons

Front 4

What CCR chemokine and chemokine receptor are associated with TH2 responses?

Back 4

CCR4 (MIP-1) --> CCL17 (TARC), CCL22

Skin homing. TH2 response.

Front 5

What CCR chemokine and chemokine receptor are associated with lymphocyte homing?

Back 5

CCL19/CCL21

CCR7

for T cell (non follical) regions of LN

Front 6

What decoy receptor protects against cancer metastasis and protects against malaria when mutated?

Back 6

DARC (Duffy)

Front 7

What CCR chemokine receptor is associated with eosinophil chemotaxis?

Back 7

CCR3 (eosinophil chemotaxis) --> binds CCL11 (Eotaxin)

Front 8

What CCR chemokine receptor is associated with T cell and monocyte trafficking ?

Back 8

CCR 1, 3, 5 (T cell and monocyte trafficking)
-->
binding CCL5 (RANTES)

Front 9

What CXCR chemokine receptor is associated with neutrophil chemotaxis?

Back 9

CXCR 1, 2 --> binds CXCL8 (IL8)

Front 10

Name the location that P selectins are expressed and their ligands?

Back 10

Location: platelets, weibel palade bodies, activated endothelium

Binds sialyl lewis X, PGSL

Brings PMN, Tcells, monos

Front 11

Name the location that E selectins are expressed and their ligands?

Back 11

Location: activated endothelium

Ligand: ESL-1, CD15 (Lewis X) , PGSL, Sialyl Lewis X (CD15s)

Brings T cells to sites of inflammation

Front 12

Name the location that L selectins are expressed and their ligands?

Back 12

Location: naive lymphocytes, leukocytes

Binds: MADCAM-1 (neutrophil rolling), GlyCAM (homing to lymph nodes), CD34 (stem cell), sialyl lewis X

Front 13

Name the B1 intergrins?

Back 13

a4B1 (VLA4) --> bind VCAM

Homing to sites of infection
Neuronal homing (used as MS treatments)

Front 14

Name the B2 intergrins?

Back 14

aLB2 (LFA-1, CD11a/CD18) on all leukocytes --> ICAM1; rhinovirus binds here

aMB2 (MAC-1, CD11b/CD18, CR3/iC3B) on macrophages --> binds ICAM1

AxB2 (CD11c/CD18, CR4/iC3B) on conventional DCs --> ICAM-3, C3dg

Front 15

What intergrin is associated with Glanzmanns thrombasthenia?

Back 15

aVB3

GIIBIII3a is defective.

Front 16

What are the B4 integrins?

Back 16

a4B7 --> binds MadCAM

gut homing

Front 17

What does PECAM (CD31) binds?

Back 17

PECAM (CD31) important for diapedesis.

Front 18

What are the clinical and laboratory features and gene defect in Chediak Higashi?

Back 18

Clinical: oculocutaneous albinism, mental retardation, neuropathy, mitral regurgitation, recurrent skin/sinus/pulm infections, gray silver hair (small clumps of pigment along shaft)

Lab:enlarged primary granules, neutropenia, chemotaxis defect, may develop HLH phase (accellerated lymphoma like syndrome)

Gene: LYST (CHS1) --> chemotaxis defect

Front 19

What are the clinical and laboratory features and gene defect of LAD1?

Back 19

Defect: No CD18

Clinical: delayed umbilical separation, necrotizing infections with impaired wound healing, cigarette paper scarring, gingivitis, omphelitis

Lab: elevated WBC, leukocytosis, poor chemotaxis

Flow shows no CD18

Front 20

What are the clinical and laboratory features and gene defect in Rac2?

Back 20

Defect: Rac2 deficiency

Clinical: Impaired chemotaxis

Front 21

What opens chromatin for transcription? (Histone acetylation vs deacetylation)

Back 21

histone acetylation.

Histone deacetylation is decreased in COPD.

Front 22

What is a superantigen?

Back 22

Bind Vbeta of TCR bypassing MHC:peptide

antigens that activate a large number of polyclonal T cells

Front 23

What is chromatine composed of?

Back 23

DNA wound around histones

Front 24

What is the difference between germline and somatic mutation?

Back 24

Germline - mutations passed on via reproductive cells

Somatic - cells outside reproductiive system; not passed on to next generation

Front 25

What are single nucleotide polymorphisms?

Back 25

single nucleotide in a gene in an individual is different from other individuals --> occur higher frequency than other mutations

Front 26

What is epigenetics?

Back 26

= changes in gene function that occur without a change in DNA sequence
--DNA methylation defect
--histone modification

Front 27

What is the gene/SNP associated with decreased response to albuterol?

Back 27

Gly Gly residue 16 --> Arg Arg
--> encodes B2 adrenergic R

Front 28

What SNPs increase the risk of atopy?

Back 28

filaggrin, ORMDL3, CD14 (LPS receptor), TLR7/8 (ssRNA), IL13, ADAM33

Front 29

What SNPs decrease the risk of atopy?

Back 29

CD14 (also can increase), CCR5

Front 30

What antimicrobial peptides are produced by kerotinocytes?

Back 30

Defensins, cathelicidins

(decreased in atopic dermatitis)

Front 31

How do antimicrobial peptides work?

Back 31

cationic proteins that interact with phospholipids of microbial membranes --> enter cell --> mediate antiproliferative effects

Front 32

Is fMLP located in cytoplasm or cell surface? What does it do?

Back 32

cell surface

PAMP for bacteria

Front 33

Is CARD located in cytoplasm or cell surface? What does it do?

Back 33

cytoplasm

--part of inflammosome
recognizes viral RNA
--induces type 1 interferons

Front 34

Is RIG-I, MDA5 located in cytoplasm or cell surface? What does it do?

Back 34

are CARD proteins

MDA5 complexes wit RIGI

cytoplasm

--part of inflammosome
recognizes viral RNA
--induces type 1 interferons

Front 35

Is CLR (c-type lectin receptor) located in cytoplasm or cell surface? What does it do?

Back 35

cell surface, secreted

anti-fungal - calcium dependent carbhydrate binding domains (mannose binding lectin)

Front 36

Is MBL (mannose binding lectin) located in cytoplasm or cell surface What does it do?

Back 36

cell surface, secreted

anti-fungal - calcium dependent carbhydrate binding domains (mannose binding lectin)

Front 37

Is macrophage mannose receptor located in cytoplasm or cell surface?

Back 37

cell surface, secreted

anti-fungal - calcium dependent carbhydrate binding domains (mannose binding lectin)

Front 38

Are the NLR/Notch/NALPS located in cytoplasm or cell surface? What does it do?

Back 38

cytoplasm

--secrete IL1b, IL18
--part of inflammasome

Front 39

What does NOD2 do?

Back 39

binds muramyl dipeptide

in NLR/Notch/NALPS family

cytoplasm

--secrete IL1b, IL18
--part of inflammasome

Mutated in IBD

Front 40

Is Dectin1 in the cell surface or cytoplasm? What does it do?

Back 40

cell surface

recognizes fungal wall Beta-glucan

defects lead to mucocutaneous candidiasis

Front 41

What caspases does the inflammasome activate? What gets produced?

Back 41

caspase 1, 5

IL1, IL18 produced

Front 42

What mutation is in muckle wells, NOMID/CINCA, familial cold urticaria syndrome?

Back 42

activating mutation in CIAS1 --> gene for inflammasome

Front 43

Which TLR are intracellular

Back 43

TLR 3, 7, 8, 9

Front 44

What does TLR1 bind?

Back 44

TLR1 with TLR2 binds mycobacteria

Front 45

What does TLR2 bind?

Back 45

TLR2 with TLR6 binds HSP70, fungus, mycoplasma, TB

Front 46

What does TLR3 bind?

Back 46

dsRNA (HSV, influenza)

Front 47

What does TLR4 bind?

Back 47

LPS (gram -)
gram+
RSV
peptidoglycan
HSP70, HSP90
aspergillus

Front 48

What does TLR5 bind?

Back 48

flagellen
associated with legionaires

Front 49

What does TLR6 bind?

Back 49

with TLR2

HSP70
fungus
mycoplasma
TB

Front 50

What does TLR7 bind?

Back 50

bind ssRNA

deficiency may also lead to susceptibility to HSV encephelitits

Front 51

What does TLR8 bind?

Back 51

bind ssRNA

deficiency may also lead to susceptibility to HSV encephelitits

Front 52

What does TLR9 bind?

Back 52

unmethylated CPG

deficiency may also lead to susceptibility to HSV encephelitits

Front 53

What does TLR11 bind?

Back 53

profillin

associated with toxo gondii

Front 54

Which TLRs are My88 independent?

Back 54

TLR3

TLR4 can be.

Front 55

What defects are assocated with HSV encephelitis?

Back 55

TLR3, 7, 8, 9
UNC93B
TRAF3

Front 56

What TLR defects are assocated with adrenal insufficiency?

Back 56

TLR 2, 4

Front 57

What defect is associated with crohns disease?

Back 57

NOD2

Front 58

What TLR defect is associated with aspergillosis?

Back 58

TLR4

Front 59

What TLR defect is associated with TB?

Back 59

TLR2, TLR6

Front 60

How does imiquimod (and resiquimod) work?

Back 60

imiquimod TLR7 agonist
resiquimod - TLR7, 8 agonists

--> interferon production, antiviral