Search on: UROPORPHYRINOGEN CARBOXY-LYASE 
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Descriptor English:   Uroporphyrinogen Decarboxylase 
Descriptor Spanish:   Uroporfirinógeno Descarboxilasa 
Descriptor Portuguese:   Uroporfirinogênio Descarboxilase 
Synonyms English:   Uroporphyrinogen III Decarboxylase
Carboxy-Lyase, Uroporphyrinogen
Decarboxylase, Uroporphyrinogen
Decarboxylase, Uroporphyrinogen III
Uroporphyrinogen Carboxy Lyase
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
DF deficiency 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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