Gluconeogenesis Disorders

Question 1

Biotin deficiencies will affect which enzymes of gluconeogenesis?

Answer 1

pyruvate carboxylase step

Propionyl-COA -> succinyl CoA blocked (one step requires B7 cofactor)

Question 2

What is a biotinidase for and what happens if you don’t have it?

Answer 2

Biotinidase recycles biotin in cells

If you are deficient, you will have B7 deficiency which affects gluconeogenesis

Question 3

How does EtOH metabolism affect gluconeogenesis?

Answer 3

increased NADH (since it is more reduced than pyruvate) in the cell will prevent malate to oxaloacetate step in the cytoplasm.