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Descriptor English: Uroporphyrinogen III Synthetase
Descriptor Spanish: Uroporfirinógeno III Sintetasa
Descriptor uroporfirinógeno III sintetasa
Entry term(s) cosintasa
uroporfirinógeno III cosintetasa
uroporfirinógeno III sintasa
uroporfirinógeno isomerasa
Scope note: Enzima que cataliza la ciclación del hidroximetilbilano para dar lugar a UROPORFIRINÓGENO III y agua. Es la cuarta enzima en la vía biosintética de la enzima 8 del HEME y está codificada por el gen UROS. La mutación del gen UROS da lugar a PORFIRIA ERITROPOYÉTICA CONGÉNITA.
Descriptor Portuguese: Uroporfirinogênio III Sintetase
Descriptor French: Uroporphyrinogen III synthetase
Entry term(s): Cosynthase
Cosynthetase, Uroporphyrinogen III
Isomerase, Uroporphyrinogen
Synthase, Uroporphyrinogen-III
Synthetase, Uroporphyrinogen III
Uroporphyrinogen III Cosynthetase
Uroporphyrinogen III Synthase
Uroporphyrinogen Isomerase
Uroporphyrinogen-III Synthase
Tree number(s): D08.811.520.241.300.950
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D014576
Scope note: An enzyme that catalyzes the cyclization of hydroxymethylbilane to yield UROPORPHYRINOGEN III and water. It is the fourth enzyme in the 8-enzyme biosynthetic pathway of HEME, and is encoded by UROS gene. Mutations of UROS gene result in CONGENITAL ERYTHROPOIETIC PORPHYRIA.
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, Erythropoietic
Registry Number: EC 4.2.1.75
CAS Type 1 Name: Hydroxymethylbilane hydro-lyase (cyclizing)
Previous Indexing: Isomerases (1971-1974)
Porphobilinogen (1973-1974)
Public MeSH Note: 2005; see UROPORPHYRINOGEN-III SYNTHASE 1998-2004, see UROPORPHYRINOGEN III SYNTHASE 1991-1997, see HYDRO-LYASES 1985-1990, see ISOMERASES 1975-1984
History Note: 2005 (1975)
Related: Porphyria, Erythropoietic MeSH
DeCS ID: 14978
Unique ID: D014576
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
Uroporphyrinogen III Synthetase - Preferred
Concept UI M0022407
Scope note An enzyme that catalyzes the cyclization of hydroxymethylbilane to yield UROPORPHYRINOGEN III and water. It is the fourth enzyme in the 8-enzyme biosynthetic pathway of HEME, and is encoded by UROS gene. Mutations of UROS gene result in CONGENITAL ERYTHROPOIETIC PORPHYRIA.
Preferred term Uroporphyrinogen III Synthetase
Entry term(s) Cosynthase
Cosynthetase, Uroporphyrinogen III
Isomerase, Uroporphyrinogen
Synthase, Uroporphyrinogen-III
Synthetase, Uroporphyrinogen III
Uroporphyrinogen III Cosynthetase
Uroporphyrinogen III Synthase
Uroporphyrinogen Isomerase
Uroporphyrinogen-III Synthase



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