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Descriptor English: Uroporphyrinogen Decarboxylase
Descriptor Spanish: Uroporfirinógeno Descarboxilasa
Descriptor uroporfirinógeno descarboxilasa
Entry term(s) uroporfirinógeno carboxiliasa
Scope note: Enzima que cataliza la descarboxilación del UROPORFIRINÓGENO III a coproporfirinógeno III mediante la conversión de cuatro grupos acetato en cuatro grupos metilo. Es la quinta enzima en la vía biosintética de la enzima 8 del HEMO. Algunas formas de PORFIRIA cutánea son el resultado de la deficiencia de esta enzima, como la PORFIRIA CUTÁNEA TARDÍA y la PORFIRIA HEPATOERITROPOYÉTICA.
Descriptor Portuguese: Uroporfirinogênio Descarboxilase
Descriptor French: Uroporphyrinogen decarboxylase
Entry term(s): Carboxy-Lyase, Uroporphyrinogen
Decarboxylase, Uroporphyrinogen
Decarboxylase, Uroporphyrinogen III
Uroporphyrinogen Carboxy Lyase
Uroporphyrinogen Carboxy-Lyase
Uroporphyrinogen III Decarboxylase
Tree number(s): D08.811.520.224.125.900
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D014575
Scope note: 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.
Annotation: /defic permitted but consider also PORPHYRIA CUTANEA TARDA and HEPATOERYTHROPOIETIC 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
DF deficiency
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
Registry Number: EC 4.1.1.37
CAS Type 1 Name: Uroporphyrinogen-III carboxy-lyase
Previous Indexing: Carboxy-Lyases (1974-1975)
Porphyrinogens (1975)
Porphyrins (1974)
Public MeSH Note: 91; was see under CARBOXY-LYASES 1976-90
History Note: 91(76); was see under CARBOXY-LYASES 1976-90
DeCS ID: 14977
Unique ID: D014575
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1991/01/01
Date of Entry: 1975/07/25
Revision Date: 2013/07/09
Uroporphyrinogen Decarboxylase - Preferred
Concept UI M0022406
Scope note 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.
Preferred term Uroporphyrinogen Decarboxylase
Entry term(s) Carboxy-Lyase, Uroporphyrinogen
Decarboxylase, Uroporphyrinogen
Decarboxylase, Uroporphyrinogen III
Uroporphyrinogen Carboxy Lyase
Uroporphyrinogen Carboxy-Lyase
Uroporphyrinogen III Decarboxylase



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