G6PDH Deficiency

Question 1

What does a G6PDH deficiency result mean in terms of ROS damage?

Answer 1

G6PDH is an enzyme required for the pentose phosphate pathway, it is involved in the production of NADPH.

NADPH is used by glutathione reductase to regenerate GSH (reduced glutathione- something that defends against oxidative stress).

If there is less G6PDH, there is less NADPH and thus there is less GSH to protect against damage from ROS.

Question 2

If the ROS arent being dealt with by GSH ( because of a G6PDH deficiency), what can happen?

Answer 2

Lipid peroxidation; cell membrane damage

Protein damage; aggregates of cross linked haemoglobin

Question 3

What ais a clinical sign of a G6PDH deficiency?

Answer 3

Heinz bodies

Question 4

What are heinz bodies?

Answer 4

Dark staining within RBCs resulting from precipitated Haemoglobin

Question 5

What are the consequences of heinz bodies?

Answer 5

Bind to cell membranes altering rigidity of RBC

Increased mechanical stress when cells squeeze through small capillaries

Question 6

What organ is responsible for removing heinz bodies?

Answer 6

Spleen

Produces ‘blister cells’