Porphobilinogen


Porphobilinogen is an organic compound that occurs in living organisms as an intermediate in the biosynthesis of porphyrins, which include critical substances like hemoglobin and chlorophyll.
The structure of the molecule can be described as molecule of pyrrole with sidechains substituted for hydrogen atoms at positions 2, 3 and 4 in the ring ; respectively, an aminomethyl group, an acetic acid group, and a propionic acid group.

Biosynthesis

In a early step of the porphyrin biosynthesis pathway, porphobilinogen is generated from aminolevulinic acid by the enzyme ALA dehydratase.

Metabolism

Next in the pathway towards porphyrins, four molecules of porphobilinogen are combined into hydroxymethyl bilane by the enzyme porphobilinogen deaminase:

Pathologies

causes an increase in urinary porphobilinogen.