Try it
Biochemistry · Fatty acid oxidation: odd-chain fatty acids
Sample itemThe final round of beta-oxidation of an odd-chain fatty acid yields propionyl-CoA, which is converted to succinyl-CoA. Which cofactors are needed, in order of the reactions?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Biotin for propionyl-CoA carboxylase, then adenosylcobalamin for methylmalonyl-CoA mutase
Propionyl-CoA is carboxylated to D-methylmalonyl-CoA by biotin-dependent propionyl-CoA carboxylase, racemised to the L form, and then rearranged to succinyl-CoA by vitamin B12-dependent methylmalonyl-CoA mutase. Because succinyl-CoA can enter gluconeogenesis, the odd-chain fatty acids are the only fatty acids with a small glucogenic contribution.
Option by option
A. Tetrahydrofolate, then lipoic acid
Tetrahydrofolate and lipoic acid are not cofactors for these reactions.
B. Biotin for propionyl-CoA carboxylase, then adenosylcobalamin for methylmalonyl-CoA mutase (correct)
Biotin acts on the carboxylation step and B12 on the mutase step, in that order.
C. Thiamine pyrophosphate, then pyridoxal phosphate
Thiamine and pyridoxal phosphate are not used in this pathway.
D. Adenosylcobalamin for propionyl-CoA carboxylase, then biotin for methylmalonyl-CoA mutase
The order is reversed: the carboxylase needs biotin and the mutase needs B12.
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…