Hydroxymethylbilane Synthase [hydroxymethylbilansynthasa]
- Terms
-
hydroxymetylbilansyntáza
porfobilinogendeaminasa
porfobilinogendeamináza
-
Hydroxymethylbilane Synthetase
Porphobilinogen Ammonia-Lyase
Porphobilinogen Deaminase
Pre-uroporphyrinogen Synthetase
Preuroporphyrinogen Synthetase
Uroporphyrinogen I Synthase
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
- DUI
- D011163 MeSH Browser
- CUI
- M0017332
- CAS
- Porphobilinogen ammonia-lyase (polymerizing)
- Previous indexing
- Ammonia-Lyases (1973-1974); Porphobilinogen (1973-1974)
- History note
- 98(75)
- Public note
- 98; see PORPHOBILINOGEN DEAMINASE 1991-97, see AMMONIA-LYASES 1975-90
Combination
- deficiency
- Porphyria, Acute Intermittent
Allowable subheadings
- AD
- administration & dosage
- AE
- adverse effects
- AN
- analysis
- AI
- antagonists & inhibitors
- BI
- biosynthesis
- BL
- blood 1
- CF
- cerebrospinal fluid
- CS
- chemical synthesis
- CH
- chemistry 2
- CL
- classification
- DE
- drug effects
- EC
- economics
- GE
- genetics 10
- HI
- history
- IM
- immunology
- IP
- isolation & purification 1
- ME
- metabolism 2
- PK
- pharmacokinetics
- PD
- pharmacology
- PH
- physiology
- PO
- poisoning
- RE
- radiation effects
- ST
- standards
- SD
- supply & distribution
- TU
- therapeutic use
- TO
- toxicity
- UL
- ultrastructure
- UR
- urine
HEMC protein, Arabidopsis Chemical MeSH Browser
hemC protein, E coli Chemical MeSH Browser