Mmg 522: Lecture #18-20

Front 1

HIV receptors

Back 1

CD4 is the receptor for HIV gp120

chemokine receptors CCR5 on macrophages & DC

Chemokine receptor CXCR4 (expressed on T cell) function as HIV coreceptors

Front 2

HIV tropism

Back 2

Determined by the gp120 variant

M-tropic: requires low CD4 and CCR5.

T-tropic: requires high CD4 and CXCR4

Front 3

regimen of HIV inhibitors that include: nuceloside reverse transcriptase (RT), protease inhibitor, non-nucleoside RT inhibitor

Back 3

HAART

Front 4

Defect in early component C1-C4 would increase susceptibility to?

Back 4

encapsulated bacteria infection

Front 5

Defect in late component C5-C9 would increase susceptibility to?

Back 5

gram-negative bacteria infection

Front 6

Phagocyte deficiency is susceptible to?

B cell & Ab deficiency?

Back 6

Pyogenic bacteria infection.

recurrent bacteria infection

Front 7

T-B+

Back 7

SCID, X-linked

Front 8

T-B-

Back 8

Adenosine deaminase deficiency (ADA)
Purine nucleoside phosphoyrlase (PNP)
RAG deficiency

Front 9

T+B-

Back 9

Omenn syndrome

Front 10

T+B+

Back 10

Bare lymphocyte syndrome (BLS)

Front 11

Most common complement deficiency that is asymptomatic.

Back 11

C2

Front 12

Most sever, susceptible to infection and fatal in early life complement deficiency

Back 12

C3

Front 13

Susceptible to gram-negative bacteria infection (Neisseria) complement deficiency

Back 13

C9

Front 14

High risk of developing immune disease complement deficiency

Back 14

C4 &C2

Front 15

Complement controller deficiency

Back 15

C1 esterase inhibitor

GPI

PNH

CD59

Front 16

Localized edema complement controller deficiency

Back 16

C1 esterase inhibitor

Front 17

GPI complement controller deficiency

Back 17

Spontaneous lysis of RBC

Front 18

PNH complement controller deficiency

Back 18

Developed by somatic DNA mutation in an enzyme essential for GPI production, can develop AML, and hemolysis in kidney at night

Front 19

CD59 complement controller deficiency

Back 19

complement regulatory protein that blocks the formation of membrane attack complex (MAC), deficiency causes RBC lysis.

Front 20

LAD=leukocyte adhesion deficiency

Back 20

A defect in the B-subunit of integrins on WBC

faulty integrins on migrating WBCs don't allow for adhesion where it is needed.

Defect in common B-subunit of integrins on WBC.

T cells don't use B subunit for migration, so patients have normal T cell function.

Symptoms include high WBC counts, no pus formation, and ineffective wound healing.

Newborns have delayed umbilical cord separation (first clinical sign). No classical signs during severe infection

Front 21

Defective in respiratory burst (phagocyte deficiency)

macrophages are recruited by cytokine secretion, but they cannot kill anything so the body keeps recruiting more.

Back 21

chronic granulomatous disease (CGD)

Front 22

Defective in degranulation and killing in phagocytes (phagocyte deficiencey)

Cells cannot break down vesicles, lysosomes cannot fuse with phagosome to kill phagocytosed microbes.

Back 22

Chediak-Higashi Syndrome

Front 23

Why is it dangerous for patients have defects in IFN-gamma receptor deficiency to receive BCG immunizations?

Back 23

Patients with IFN-gamma receptor should not receive BCG immunization bc it can cause a letal BCG infection

Front 24

Defect in IFN-gamma

Back 24

Leukocytes have chemokine receptors so they can follow chemokine signals to reach infection. If there is integrin signaling defect, they cannot go through via diapedesis and migrate to tissues.

Front 25

Syndrome caused by lack of thymus and parathyroid

Back 25

DiGeorge syndrome

Front 26

Defect in Fas, FasL or capase 10, inc. number of CD4- CD8- T cells

Back 26

ALPS-autoimmune lymphoproliferative syndrome

It is dominant and recessive bc fas mutation is aut. dominant, but caspase-10 is recessive

Front 27

Deficiency in signal transduction, leads to defective T cell function and impaired B cell function, CD8+ T cells are absent

Causes SCID-like clinical features

Back 27

ZAP-70

Front 28

T cell deficiency, impaired Th1 (defect in Th cell producing TFN-gamma)

So Th2 becomes dominant

Back 28

Hyper-IgE

Front 29

Defect in B cell class switching due to mutation in CD40L, CD40, or CD40-CD40L signaling

No germinal center formation

Back 29

Hyper-IgM

Front 30

Bruton's agammaglobulinemia, x-linked infantile agammaglobulinemia, or x-linked hypogammaglobulinemia.

caused by mutation of btk (B cell tyrosine kinase) that blocks maturation from preB

virtual absense of B cells can very low level of all Ig.

Back 30

XLA

Front 31

What are ths SCID-like multisystem disorders?

Back 31

ZAP-70 mutation
Wiskott-Aldrich syndrome
Ataxia Telangiectasia (AT)

Front 32

Baring no MHC II (due to mutation in TAP)

results in higher CD8+

Back 32

BLS Bare lymphocyte syndrome

Front 33

What SCID disorder cannot be treated with bone marrow transplant?

Back 33

Omenn Syndrome (T+B-)

Front 34

Which SCID counts for almost half of the total SCID cases?

Back 34

X-linked SCID (T-B+) no T cell, normal B cell with no function

Caused by mutation of gamma-chain cytokine receptor including IL-2, 4, 7, 9, 15.

Front 35

SCID-like multisystem disorder, caused by mutation in WASP protein that exists in all hematopoietic stem cells and binds to adapters and probably cytoskeleton components.

Back 35

Wiskott-Aldrich Syndrome:

Front 36

SCID-like multisystem disorder caused by autosomal recessive mutation in ATM, a protein involved in DNA repair in all cells.

Back 36

AT: Ataxia Telangiectasia

Front 37

Which molecules on CD4+ T cells are used by HIV to enter T cells?

On macrophages?

Back 37

T cells: CD4 receptor, CXCR4 is chemokine receptor that functions as HIV coreceptor

Macrophages: CCR5 is chemokine receptor that functions as HIV coreceptor

Front 38

How do we monitor HIV patients?

Back 38

Flow cytometry to count CD4

Normal CD4:CD8 ratio is 2:1

In HIV, ratio is 1:2