Porphobilinogen (PBG) 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. Porphobilinogen can be described as a pyrrole with substituents at positions 2, 3 and 4. These substituents are respectively, an aminomethyl group −CH2−NH2, a carboxymethyl group −CH2−COOH, and a carboxyethyl group −CH2−CH2−COOH.
Biosynthesis In an early step of the porphyrin biosynthesis pathway, porphobilinogen is generated from aminolevulinic acid (ALA) 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 Acute intermittent porphyria causes an increase in urinary porphobilinogen.
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