Biochem24

Question 1

A deficiency in NADPH oxidase is seen in what disease?

Answer 1

Chronic Granulomatous DZ

Question 2

What are patients with Chronic Granulomatous disease at risk of?

Answer 2

Infection by catalase-positive species (e.g., S. aureus, Aspergillus) because they neutralized their own H2O2, leaving WBCs without ROIs for fighting infection

Question 3

What keeps glutathione reduced?

Answer 3

NADPH

Question 4

What does glutathione do?

Answer 4

Detoxifies free radicals and peroxides

Question 5

What is the result of a Glucose-6-phosphate dehydrogenase deficiency?

Answer 5

DEcreased NADPH in RBCs –> Hemolytic Anemia

Question 6

What is the mode of inheritance for G6PD deficiency?

Answer 6

X-linked recessive

Question 7

What is the most common human enzyme deficiency?

Answer 7

G6PD deficiency

• more common in blacks
• INcreases malarial resistance

Question 8

What are Heinz bodies?

Answer 8

Oxidized Hemomoglobin precipitated within RBCs

Question 9

What are Bite Cells?

Answer 9

Result from the phagocytic removal of Heinz bodies by splenic macrophages

“Bite into some Heinz ketchup”

Question 10

What is the treatment for G6PD deficiency?

Answer 10

Splenectomy