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.

Heme synthesis—note that some reactions occur in the cytoplasm and some in the mitochondrion (yellow)

Older sources categorize it under .

References

Further reading

es:HMBS

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