Try it
Biochemistry · Vitamins and minerals: pyridoxal phosphate and sideroblastic anaemia
Sample itemA man with tuberculosis on long-term isoniazid develops microcytic hypochromic anaemia with ring sideroblasts in the marrow and a raised serum ferritin. Which reaction in haem synthesis is impaired?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Glycine condensing with succinyl-CoA to form delta-aminolevulinate
Isoniazid depletes pyridoxal phosphate, the cofactor of ALA synthase, the first and regulated step of haem synthesis. Iron then accumulates in mitochondria, giving ring sideroblasts. Pyridoxine supplementation prevents this complication.
Option by option
A. Glycine condensing with succinyl-CoA to form delta-aminolevulinate (correct)
Correct. ALA synthase needs pyridoxal phosphate, which isoniazid depletes.
B. Formation of uroporphyrinogen III from hydroxymethylbilane
Uroporphyrinogen III synthase does not depend on pyridoxal phosphate.
C. Condensation of two delta-aminolevulinate molecules to porphobilinogen
ALA dehydratase is a zinc enzyme inhibited in lead poisoning and does not need pyridoxal phosphate.
D. Insertion of iron into protoporphyrin
Ferrochelatase is inhibited by lead and does not depend on pyridoxal phosphate.
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…