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Descriptor English:   Uroporphyrinogen III Synthetase 
Descriptor Spanish:   Uroporfirinógeno III Sintetasa 
Descriptor Portuguese:   Uroporfirinogênio III Sintetase 
Synonyms English:   Cosynthetase, Uroporphyrinogen III
Isomerase, Uroporphyrinogen
Synthase, Uroporphyrinogen-III
Synthetase, Uroporphyrinogen III
Uroporphyrinogen III Synthase
Cosynthase
Uroporphyrinogen III Cosynthetase
Uroporphyrinogen Isomerase
Uroporphyrinogen-III Synthase  
Tree Number:   D08.811.520.241.300.950
Definition English:   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. 
Entry Combination - descr/qualif English:   Uroporphyrinogen III Synthetase/deficiency use Porphyria, Erythropoietic
 
See Related English:   Porphyria, Erythropoietic
 
History Note English:   2005 (1975) 
Allowable Qualifiers English:  
AD administration & dosage AE adverse effects
AN analysis AI antagonists & inhibitors
BI biosynthesis BL blood
CF cerebrospinal fluid CS chemical synthesis
CH chemistry CL classification
CT contraindications DE drug effects
EC economics GE genetics
HI history IM immunology
IP isolation & purification ME metabolism
PK pharmacokinetics PD pharmacology
PH physiology PO poisoning
RE radiation effects SE secretion
ST standards SD supply & distribution
TU therapeutic use TO toxicity
UL ultrastructure UR urine
Record Number:   14978 
Unique Identifier:   D014576 

Occurrence in VHL:
 

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