Immuno Path V - Immuno Def. cont'd

Question 1

What genetic defect is commonly seen in Wiskott-Aldrich Syndrome?

Answer 1

X-linked disorder of male infants
defect in WASP gene responsible for signal transduction and cytoskeleton maitenace.

**Lack of CD43 cytoskeleton glycoprotein.

Question 2

What is a clinical sign of Wiskott-Aldrich syndrome?

Answer 2

Impairs both cellular and humoral.

Lack of response to polysaccharide Ag in bacterial cell walls due to low IgM.

Question 3

A CD4 count of what indicates full blown AIDS?

Question 4

What cellular HIV component binds to CD4 cells?

Answer 4

Gp120 on the virus binds to CD4 molecule CCR5 (macrophages) and CxCR4 (T cells)

Then taken up by gp 41 expression.

Question 5

This HIV gene is impairative for its infectivity

Answer 5

nef

Question 6

What serum factor will be found in patients suffering from amyloidosis?

Answer 6

Serum amyloid-P

Question 7

Amyloid will stain in what manner?

Answer 7

Congo red with polarized light will show up apple green!

Question 8

What configuration are the fibrils in when having amyloidosis?

Answer 8

B-Pleated sheets.

Question 9

Amyloid light chain type is derived from what type of cells?

Answer 9

Derived from plasma cells with frequent involvement of the heart, GIT, peripheral nerves, skin and tongue.

Question 10

Amyloid associated type Amyloidosis is related to what?

Answer 10

Derived from serum amyloid associated protein (SAA) which is an acute phase protein from the liver.

Most commonly seen in RA, IBD, and Hodgkins lymphoma.

Question 11

What does ATTR (Amyloid transthyretin) cause?

Answer 11

Amyloid transthyretin related to senile cardiac amyloid.

Question 12

What does Amyloid beta cause?

Answer 12

Found in Alzheimers

Question 13

AB 2 microglobulin protein (Amyloid) is found in what?

Answer 13

Normal component of MHC class I