Porphobilinogen deaminase (HMBS)
Cofactors used
Clinical / pharmacological. Acute intermittent porphyria: abdominal pain, neuropsychiatric signs, no photosensitivity
What it does, reaction by reaction
Heme Biosynthesis (Porphyrin Pathway) Amino Acid & Nitrogen Metabolism · Mitochondrion + cytosol
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
No regulation recorded for this enzyme in the source documents.
Recent literature
Europe PMC · from cache · sorted by publication date
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1
Limited benefit of liver transplantation in a boy with biallelic severe deficiency of hydroxymethylbilane synthase and review of prior reported cases.
Araque M, Ma CD, Jordan A, Faust D, McGuire L, Bonkovsky HL. · 2026-06-27
unreviewed -
2
Unraveling the alkali tolerance of microcystinase A within alkaline algal bloom scenarios.
Zhang W, Shao L, Xiang X, Cheng C, Wu Y, Kou X, Yu J, Xiong Z, Guo X, Feng L. · 2026-05-25
unreviewed -
3
Improving resilience in human milk banking: workshop proceedings, 11th November, London UK.
Shenker N, Hatia A, Griffin S, Chandler-Maini G, Bailie L, Barnett D, Savage E, D'Souza A, Dowling C, Gane T,… · 2026-08-12
unreviewed -
4
Pharmacokinetic and pharmacokinetic/pharmacodynamic (PK/PD) considerations of ceftaroline and ceftobiprole for the treatment of Staphylococcus aureus infections: a systematic review.
Genovese C, Colaneri M, Sguazzini E, Maddalena D, Abdul-Aziz MH, Sime FB, Soriano A, Gori A, Roberts JA, Ulld… · 2026-08-10
unreviewed -
5
Assessing post-mortem interval in cats (Felis catus) through RNA degradation analysis in liver tissue.
Botta L, Divari S, Pregel P, Ricci E, Ressel L, Scaglione FE. · 2026-08-09
unreviewed -
6
Population Pharmacokinetics of Intravenous Vitamin C in Adults with Sepsis: A Sub-study of the LOVIT Trial.
Kanji S, Hernández-Mitre MP, Blain H, Adhikari NKJ, Lamontagne F, Battista MC, Ménard J, Sprague S, Masse MH,… · 2026-06-22
unreviewed -
7
Threonine and glutamine in ovo supplementation affects post-hatch development and expression of genes related to the first line of defense of the intestinal mucosa in broiler chickens.
Santos WS, Sousa AV, Gomes DS, Marinho GF, Oliveria TJ, Givisiez PEN, Oliveira CJB, Malheiros RD, Filho ALBM. · 2026-04-22
open access unreviewed -
8
Predictive performance of an antibiotic precision dosing software program in critically ill adults with infection.
Williams P, Cotta MO, Wilks K, Farkas A, Spelman T, Roberts JA. · 2026-07-01
unreviewed -
9
Immigrant Fathers' Perceptions of Human Milk Banks and Pasteurized Donor Human Milk in the United States: A Qualitative Study.
Obeng CS, Jackson F, Ayiku H, Salem S, Gonzalez Casanova I, Perry CA, Byrd KA. · 2026-07-17
unreviewed -
10
Informal Human Milk Sharing: The Experiences and Practices of Informal Human Milk Sharing in Ireland.
Vickers N, Matthews A, Paul G. · 2026-07-01
unreviewed
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.