Search on: UROPORPHYRINOGEN ISOMERASE 
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Descriptor English:   Uroporphyrinogen III Synthetase 
Descriptor Spanish:   Uroporfirinógeno III Sintetasa 
Descriptor Portuguese:   Uroporfirinogênio III Sintetase 
Synonyms English:   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 DU diagnostic use
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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