Porphobilinogen deaminase

Porphobilinogen deaminase (or hydroxymethylbilane synthase) is an enzyme involved in the third step of the metabolism of porphyrin, converting porphobilinogen into hydroxymethyl bilane. The enzyme has the unique cofactor dipyrromethane. Defective activity of this enzyme can lead to the disorder Acute intermittent porphyria.



Older sources categorize it under.