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Where do superantigens bind?
V-beta chain of TCR
What does the SEB superantigen from Staph aureus cause?
Food poisoning.
What does the SEC2 superantigen from Staph aureus cause?
Food poisoning
What does the TSST superantigen from Staph aureus cause?
Toxic shock
What does the SPE-C superantigen from Strep pyogenes cause?
Toxic shock
What do CD1 molecules bind?
Lipids
What 2 cell types characteristically recognize antigens presented by CD1 molecules?
NKT cells
gamma-delta T cells
How many amino acids are contained in peptides presented by MHC class I molecules?
8-11
How many amino acids are contained in peptides presented by MHC class II molecules?
10-30
What route of administration is more immunogenic, subcutaneous or intravenous?
subcutaneous
What route of administration is more immunogenic, subcutaneous or intragastric?
subcutaneous
What region of MHC class I molecule interacts with CD8?
alpha-3
What region of MHC class II molecule interacts with CD4?
beta-2
What 3 cytokines characteristically induce MHC class I expression?
IFN-alpha
IFN-beta
IFN-gamma
What 1 cytokine characteristically induces MHC class II expression?
IFN-gamma
What chromosome contains the MHC genes?
Chromosome 6 (short arm)
What chromosome contains the beta-2 microglobulin gene?
Chromosome 15
What role does HLA E perform?
Recognition by NK cells
What role does HLA F perform?
Not stated (localizes to ER and golgi)
What role dose HLA G perform?
Not stated (on fetal derived placental cells)
What role does HLA H perform?
Utilized in iron metabolism.
What 4 proteins interact with newly formed MHC class I molecules to sequester them in the ER?
Calnexin
Calreticulin
Erp 57
Tapasin
What is the proteosome?
cytoplasmic multi-subunit proteinase that degrades proteins prior to transport into the ER
What are 2 examples of proteasome subunits?
LMP 7
LMP 2

In what chromosome are these subunits encoded?
Chromosome 6 (within the MHC class II locus)
What is TAP?
"Transporter Associated with antigen Processing" ER multi-subunit protein that utilizes energy to transport antigenic peptides into the ER
What are the 2 subunits that compose TAP?
TAP1
TAP2

In what chromosome are these subunites encoded?
Chromosome 6 (within the MHC class II locus)
What is Ii?
Invariant chain. Functions to stabilize MHC class II molecules in the ER (prior to loading with antigen).
What is CLIP?
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
What molecule removes CLIP and allows antigenic peptides to be loaded into the MHC binding cleft?
HLA-DM
What role does HLA-DM perform?
Removes CLIP from MHC class II molecules to allow antgen binding.
Is HLA-DM involved in direct antigen presentation?
No.
What proteins are mutated in MHC class I deficiency?
TAP1/TAP2
What is the mode of inheritance for MHC class I deficiency?
AR
What is the key lymphocyte enumeration finding for MHC class I deficiency?
No CD8+ T-cells.
What is the treatment for MHC class I deficiency?
Aggressive antibiotics for infection, aggressive pulmonary toilet/chest PT.

Is HSCT commonly used for MHC class I deficiency?
No.
What proteins are mutated in MHC class II deficiency?
Transcription factors for MHC class II expression: MHC2TA, RFX5, FRXAP, FRXANK
What is the mode of inheritance for MHC class II deficiency?
AR
What are clinical features of MHC class I deficiency?
Sinopulmonary infections, granulomatous skin lesions, necrobiosis lipoidica.
What are clinical features of MHC class II deficiency?
Diarrhea, hepatosplenomegaly, transaminitis, sclerosing cholangitis, pulmonary infections, meningitis
What is the key lymphocyte enumeration finding in MHC class II deficiency?
CD4 lymphopenia
What is the key quantitative immunoglobulin lab finding in MHC class II deficiency?
Hypogammaglobulinemia
What is the key pathologic finding on lymph node biopsy in MHC class II deficiency?
Absent germinal centers.
What is the treatment for MHC class II deficiency?
HSCT