Concept information
Preferred term
Hydroxymethylbilane Synthase
Type
-
mesh:Descriptor
Definition
- An enzyme that catalyzes the tetrapolymerization of the monopyrrole PORPHOBILINOGEN into the hydroxymethylbilane preuroporphyrinogen (UROPORPHYRINOGENS) in several discrete steps. It is the third enzyme in the 8-enzyme biosynthetic pathway of HEME. In humans, deficiency in this enzyme encoded by HMBS (or PBGD) gene results in a form of neurological porphyria (PORPHYRIA, ACUTE INTERMITTENT). This enzyme was formerly listed as EC 4.3.1.8
Broader concept
Entry terms
- 9036-47-9 (CAS RN)
- Hydroxymethylbilane Synthetase
- Porphobilinogen Ammonia-Lyase
- Porphobilinogen ammonia-lyase (polymerizing)
- Porphobilinogen Deaminase
- Pre-uroporphyrinogen Synthetase
- Preuroporphyrinogen Synthetase
- Uroporphyrinogen I Synthase
Allowable Qualifier(s)
- administration & dosage (Qualifier)
- adverse effects (Qualifier)
- analysis (Qualifier)
- antagonists & inhibitors (Qualifier)
- biosynthesis (Qualifier)
- blood (Qualifier)
- cerebrospinal fluid (Qualifier)
- chemical synthesis (Qualifier)
- chemistry (Qualifier)
- classification (Qualifier)
- drug effects (Qualifier)
- economics (Qualifier)
- genetics (Qualifier)
- history (Qualifier)
- immunology (Qualifier)
- isolation & purification (Qualifier)
- metabolism (Qualifier)
- pharmacokinetics (Qualifier)
- pharmacology (Qualifier)
- physiology (Qualifier)
- poisoning (Qualifier)
- radiation effects (Qualifier)
- standards (Qualifier)
- supply & distribution (Qualifier)
- therapeutic use (Qualifier)
- toxicity (Qualifier)
- ultrastructure (Qualifier)
- urine (Qualifier)
In other languages
-
French
-
9036-47-9 (CAS RN)
-
Porphobilinogen ammonia-lyase (polymerizing)
-
Porphobilinogen ammonia-lyase(polymerizing)
-
Porphobilinogène ammonium-lyase(polymérisante)
-
Porphobilinogène désaminase
-
Pré-uroporphyrinogène synthase
URI
http://data.loterre.fr/ark:/67375/JVR-H15QXDJL-L
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