Search on: UROPORPHYRINOGEN CARBOXY-LYASE 
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Descriptor English:   Uroporphyrinogen Decarboxylase 
Descriptor Spanish:   Uroporfirinógeno Descarboxilasa 
Descriptor Portuguese:   Uroporfirinogênio Descarboxilase 
Synonyms English:   Uroporphyrinogen Carboxy-Lyase  
Tree Number:   D08.811.520.224.125.900
Definition English:   An enzyme that catalyzes the decarboxylation of UROPORPHYRINOGEN III to coproporphyrinogen III by the conversion of four acetate groups to four methyl groups. It is the fifth enzyme in the 8-enzyme biosynthetic pathway of HEME. Several forms of cutaneous PORPHYRIAS are results of this enzyme deficiency as in PORPHYRIA CUTANEA TARDA; and HEPATOERYTHROPOIETIC PORPHYRIA. 
Indexing Annotation English:   /defic permitted but consider also PORPHYRIA CUTANEA TARDA and HEPATOERYTHROPOIETIC PORPHYRIA
History Note English:   91(76); was see under CARBOXY-LYASES 1976-90 
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 DF deficiency
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:   14977 
Unique Identifier:   D014575 

Occurrence in VHL:
 

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