unreviewed

Porphobilinogen deaminase (HMBS)

1 reactions · 1 pathways

Cofactors used

Dipyrromethane

Clinical / pharmacological. Acute intermittent porphyria: abdominal pain, neuropsychiatric signs, no photosensitivity

What it does, reaction by reaction

1 reactions

Heme Biosynthesis (Porphyrin Pathway) Amino Acid & Nitrogen Metabolism · Mitochondrion + cytosol

step 3 Irreversible/directional

4 porphobilinogen → hydroxymethylbilane + 4 NH₃

Converts Porphobilinogen into Hydroxymethylbilane NH3

› Notes

Porphobilinogen deaminase, also called hydroxymethylbilane synthase (HMBS), polymerizes four PBG units using a dipyrromethane cofactor covalently attached to the enzyme. The linear tetrapyrrole product is unstable and is normally consumed immediately.

Showing all 1 reactions.

What accelerates and inhibits it

Regulation is pathway-specific, so each context is listed separately

0 entries

No regulation recorded for this enzyme in the source documents.

Recent literature

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