Try it
Biochemistry · Carbohydrate metabolism: L-gulonolactone oxidase and ascorbate
Sample itemMost mammals make ascorbic acid from glucose through the uronic acid pathway, but humans and guinea pigs cannot and need it in the diet. Which enzyme is non-functional in humans?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. L-gulonolactone oxidase
The last enzyme of ascorbate synthesis, L-gulonolactone oxidase, is encoded by a pseudogene in humans, other primates and guinea pigs. Humans therefore depend on dietary vitamin C. The rest of the uronic acid pathway, which gives glucuronate for conjugation, still functions.
Option by option
A. Glucose-6-phosphate dehydrogenase
Glucose-6-phosphate dehydrogenase is the first enzyme of the pentose phosphate pathway and is not involved in ascorbate synthesis.
B. L-gulonolactone oxidase (correct)
Correct. The gene for L-gulonolactone oxidase is inactive in humans, so ascorbate cannot be made.
C. Aldose reductase
Aldose reductase forms sorbitol from glucose in the polyol pathway and is active in humans.
D. UDP-glucose dehydrogenase
UDP-glucose dehydrogenase works normally in humans and produces UDP-glucuronate used for conjugation.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
More Biochemistry questions
- Nucleotide metabolismA 4-year-old boy has intellectual disability, dystonia, self-mutilation and hyperuricaem…
- VitaminsA patient with megaloblastic anaemia and mild neurological signs has raised serum methyl…
- Amino acid metabolismA baby has a mousy odour, fair skin and developmental delay. Which enzyme is deficient?
- Glycogen storage diseasesA child has severe fasting hypoglycaemia, hepatomegaly, lactic acidosis and hyperuricaem…