Hydroxymethylbilane Synthase [hydroxymethylbilansynthasa]
topical
11
- Terms
-
hydroxymetylbilansyntáza
porfobilinogendeaminasa
porfobilinogendeamináza
-
Hydroxymethylbilane Synthetase
Porphobilinogen Ammonia-Lyase
Porphobilinogen Deaminase
Pre-uroporphyrinogen Synthetase
Preuroporphyrinogen Synthetase
Uroporphyrinogen I Synthase
Persistent link
https://www.medvik.cz/link/D011163
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
- 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 0
- AE
- adverse effects 0
- AN
- analysis 0
- AI
- antagonists & inhibitors 0
- BI
- biosynthesis 0
- BL
- blood 1
- CF
- cerebrospinal fluid 0
- CS
- chemical synthesis 0
- CH
- chemistry 2
- CL
- classification 0
- DE
- drug effects 0
- EC
- economics 0
- GE
- genetics 10
- HI
- history 0
- IM
- immunology 0
- IP
- isolation & purification 1
- ME
- metabolism 2
- PK
- pharmacokinetics 0
- PD
- pharmacology 0
- PH
- physiology 0
- PO
- poisoning 0
- RE
- radiation effects 0
- ST
- standards 0
- SD
- supply & distribution 0
- TU
- therapeutic use 0
- TO
- toxicity 0
- UL
- ultrastructure 0
- UR
- urine 0
...
Occurrences in Medvik records
D
Chemicals and Drugs
D08.811.913.225.250
3-Deoxy-7-Phosphoheptulonate Synthase
D08.811.913.225.300
Dihydropteroate Synthase
D08.811.913.225.443
Geranylgeranyl-Diphosphate Geranylgeranyltransferase
D08.811.913.225.735
3-Phosphoshikimate 1-Carboxyvinyltransferase
D08.811.913.225.825
Spermidine Synthase
D08.811.913.225.912
Spermine Synthase