Question

Porphobilinogen is converted to hydroxymethylbilane via the enzyme porphobilinogen deaminase (heme synthesis). Deficiency of this enzyme causes which condition?

1) Acute intermittent porphyria
2) Hemochromatosis
3) Sideroblastic anemia
4) Porphyria cutanea tarda

Answer 1

Porphobilinogen deaminase deficiency causes Acute intermittent porphyria, a type of hepatic porphyria affecting heme synthesis.

Step-by-step explanation:

Deficiency of the enzyme porphobilinogen deaminase, which is responsible for the conversion of porphobilinogen to hydroxymethylbilane in heme synthesis, causes the condition known as Acute intermittent porphyria.

This is a type of hepatic porphyria. Porphyrias involve a group of diseases where there is an issue with the synthesis of porphyrins, leading to an increased excretion of porphyrins or their precursors.

The synthesis of heme is a critical biological process and any disruption in the heme synthesis pathway can lead to significant health conditions. Acute intermittent porphyria is a disorder related to the liver and has to do with the body's ability to produce heme.