Acaai Board Review Chapter 1, Section 1

Front 1

Where do superantigens bind?

Back 1

V-beta chain of TCR

Front 2

What does the SEB superantigen from Staph aureus cause?

Back 2

Food poisoning.

Front 3

What does the SEC2 superantigen from Staph aureus cause?

Back 3

Food poisoning

Front 4

What does the TSST superantigen from Staph aureus cause?

Back 4

Toxic shock

Front 5

What does the SPE-C superantigen from Strep pyogenes cause?

Back 5

Toxic shock

Front 6

What do CD1 molecules bind?

Back 6

Lipids

Front 7

What 2 cell types characteristically recognize antigens presented by CD1 molecules?

Back 7

NKT cells
gamma-delta T cells

Front 8

How many amino acids are contained in peptides presented by MHC class I molecules?

Back 8

8-11

Front 9

How many amino acids are contained in peptides presented by MHC class II molecules?

Back 9

10-30

Front 10

What route of administration is more immunogenic, subcutaneous or intravenous?

Back 10

subcutaneous

Front 11

What route of administration is more immunogenic, subcutaneous or intragastric?

Back 11

subcutaneous

Front 12

What region of MHC class I molecule interacts with CD8?

Back 12

alpha-3

Front 13

What region of MHC class II molecule interacts with CD4?

Back 13

beta-2

Front 14

What 3 cytokines characteristically induce MHC class I expression?

Back 14

IFN-alpha
IFN-beta
IFN-gamma

Front 15

What 1 cytokine characteristically induces MHC class II expression?

Back 15

IFN-gamma

Front 16

What chromosome contains the MHC genes?

Back 16

Chromosome 6 (short arm)

Front 17

What chromosome contains the beta-2 microglobulin gene?

Back 17

Chromosome 15

Front 18

What role does HLA E perform?

Back 18

Recognition by NK cells

Front 19

What role does HLA F perform?

Back 19

Not stated (localizes to ER and golgi)

Front 20

What role dose HLA G perform?

Back 20

Not stated (on fetal derived placental cells)

Front 21

What role does HLA H perform?

Back 21

Utilized in iron metabolism.

Front 22

What 4 proteins interact with newly formed MHC class I molecules to sequester them in the ER?

Back 22

Calnexin
Calreticulin
Erp 57
Tapasin

Front 23

What is the proteosome?

Back 23

cytoplasmic multi-subunit proteinase that degrades proteins prior to transport into the ER

Front 24

What are 2 examples of proteasome subunits?

Back 24

LMP 7
LMP 2

In what chromosome are these subunits encoded?

Hint 24

Chromosome 6 (within the MHC class II locus)

Front 25

What is TAP?

Back 25

"Transporter Associated with antigen Processing" ER multi-subunit protein that utilizes energy to transport antigenic peptides into the ER

Front 26

What are the 2 subunits that compose TAP?

Back 26

TAP1
TAP2

In what chromosome are these subunites encoded?

Hint 26

Chromosome 6 (within the MHC class II locus)

Front 27

What is Ii?

Back 27

Invariant chain. Functions to stabilize MHC class II molecules in the ER (prior to loading with antigen).

Front 28

What is CLIP?

Back 28

The short peptide that is generated when the invariant chain is degraded by proteolytic enzymes in the MHC class II vessicle.

It occupies the antigen binding cleft in MHC class II molecule prior to antigen binding

Front 29

What molecule removes CLIP and allows antigenic peptides to be loaded into the MHC binding cleft?

Back 29

HLA-DM

Front 30

What role does HLA-DM perform?

Back 30

Removes CLIP from MHC class II molecules to allow antgen binding.

Front 31

Is HLA-DM involved in direct antigen presentation?

Back 31

No.

Front 32

What proteins are mutated in MHC class I deficiency?

Back 32

TAP1/TAP2

Front 33

What is the mode of inheritance for MHC class I deficiency?

Back 33

AR

Front 34

What is the key lymphocyte enumeration finding for MHC class I deficiency?

Back 34

No CD8+ T-cells.

Front 35

What is the treatment for MHC class I deficiency?

Back 35

Aggressive antibiotics for infection, aggressive pulmonary toilet/chest PT.

Is HSCT commonly used for MHC class I deficiency?

Hint 35

No.

Front 36

What proteins are mutated in MHC class II deficiency?

Back 36

Transcription factors for MHC class II expression: MHC2TA, RFX5, FRXAP, FRXANK

Front 37

What is the mode of inheritance for MHC class II deficiency?

Back 37

AR

Front 38

What are clinical features of MHC class I deficiency?

Back 38

Sinopulmonary infections, granulomatous skin lesions, necrobiosis lipoidica.

Front 39

What are clinical features of MHC class II deficiency?

Back 39

Diarrhea, hepatosplenomegaly, transaminitis, sclerosing cholangitis, pulmonary infections, meningitis

Front 40

What is the key lymphocyte enumeration finding in MHC class II deficiency?

Back 40

CD4 lymphopenia

Front 41

What is the key quantitative immunoglobulin lab finding in MHC class II deficiency?

Back 41

Hypogammaglobulinemia

Front 42

What is the key pathologic finding on lymph node biopsy in MHC class II deficiency?

Back 42

Absent germinal centers.

Front 43

What is the treatment for MHC class II deficiency?

Back 43

HSCT