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Descriptor English: Hydroxymethylbilane Synthase
Descriptor Spanish: Hidroximetilbilano Sintasa
Descriptor hidroximetilbilano sintasa
Entry term(s) porfobilinógeno amoniacoliasa
porfobilinógeno desaminasa
uroporfirinógeno I sintasa
Scope note: Enzima que cataliza la tetrapolimerización del PORFOBILINÓGENO monopirrólico a hidroximetilbilano, un precursor del uroporfirinógeno (UROPORFIRINÓGENOS), en varios pasos concretos. Es la tercera enzima de la vía biosintética de 8 enzimas del HEME. En humanos, la deficiencia de esta enzima codificada por el gen HMBS (o PBGD) da lugar a un tipo de porfiria neurológica (PORFIRIA AGUDA INTERMITENTE). Esta enzima fue clasificada anteriormente como EC 4.3.1.8.
Descriptor Portuguese: Hidroximetilbilano Sintase
Descriptor French: Hydroxymethylbilane synthase
Entry term(s): Ammonia-Lyase, Porphobilinogen
Deaminase, Porphobilinogen
Hydroxymethylbilane Synthetase
Porphobilinogen Ammonia Lyase
Porphobilinogen Ammonia-Lyase
Porphobilinogen Deaminase
Pre uroporphyrinogen Synthetase
Pre-uroporphyrinogen Synthetase
Preuroporphyrinogen Synthetase
Synthase, Hydroxymethylbilane
Synthase, Uroporphyrinogen I
Synthetase, Hydroxymethylbilane
Synthetase, Pre-uroporphyrinogen
Synthetase, Preuroporphyrinogen
Uroporphyrinogen I Synthase
Tree number(s): D08.811.913.225.575
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D011163
Scope note: 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
Allowable Qualifiers: AD administration & dosage
AE adverse effects
AI antagonists & inhibitors
AN analysis
BI biosynthesis
BL blood
CF cerebrospinal fluid
CH chemistry
CL classification
CS chemical synthesis
DE drug effects
EC economics
GE genetics
HI history
IM immunology
IP isolation & purification
ME metabolism
PD pharmacology
PH physiology
PK pharmacokinetics
PO poisoning
RE radiation effects
SD supply & distribution
ST standards
TO toxicity
TU therapeutic use
UL ultrastructure
UR urine
Entry Combination: deficiency:Porphyria, Acute Intermittent
Registry Number: EC 2.5.1.61
CAS Type 1 Name: Porphobilinogen ammonia-lyase (polymerizing)
Previous Indexing: Ammonia-Lyases (1973-1974)
Porphobilinogen (1973-1974)
Public MeSH Note: 98; see PORPHOBILINOGEN DEAMINASE 1991-97, see AMMONIA-LYASES 1975-90
History Note: 98(75)
Related: Porphyrias MeSH
DeCS ID: 11605
Unique ID: D011163
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1991/01/01
Date of Entry: 1974/11/19
Revision Date: 2009/07/06
Hydroxymethylbilane Synthase - Preferred
Concept UI M0017332
Scope note 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
Preferred term Hydroxymethylbilane Synthase
Entry term(s) Ammonia-Lyase, Porphobilinogen
Deaminase, Porphobilinogen
Hydroxymethylbilane Synthetase
Porphobilinogen Ammonia Lyase
Porphobilinogen Ammonia-Lyase
Porphobilinogen Deaminase
Pre uroporphyrinogen Synthetase
Pre-uroporphyrinogen Synthetase
Preuroporphyrinogen Synthetase
Synthase, Hydroxymethylbilane
Synthase, Uroporphyrinogen I
Synthetase, Hydroxymethylbilane
Synthetase, Pre-uroporphyrinogen
Synthetase, Preuroporphyrinogen
Uroporphyrinogen I Synthase



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