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Information on EC 4.1.1.37 - uroporphyrinogen decarboxylase
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4.1.1.37 uroporphyrinogen decarboxylaseIUBMB Comments
Acts on a number of porphyrinogens.
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Word Map
- 4.1.1.37
- porphyria
-
cutanea
- tarda
- heme
- erythrocyte
-
porphyrinogenic
- hexachlorobenzene
- urinary
-
hepatoerythropoietic
- porphobilinogen
- overload
-
photosensitivity
-
ferrochelatase
- protoporphyrin
- hemochromatosis
-
5-aminolaevulinate
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heptacarboxylic
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hexachlorobenzene-induced
- hypertrichosis
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phlebotomy
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erythropoietic
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delta-aminolaevulinic
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polyhalogenated
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5-aminolevulinic
- protoporphyrinogen
-
uroporphyria
- tetrapyrrole
-
sun-exposed
- blister
- siderosis
- diagnostics
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3,4,3\',4\'-tetrachlorobiphenyl
- medicine
-
hcb-induced
-
miliae
- coproporphyria
- bulla
- agriculture
-
hepta
The enzyme appears in viruses and cellular organisms
Synonyms
uroporphyrinogen decarboxylase, uro-d, porphyrinogen carboxy-lyase, uroporphyrinogen iii decarboxylase, urod protein, uroporphyrinogen-decarboxylase, uro-decarboxylase, uroporphyrinogen-iii decarboxylase, hem12p, tobacco urod, 
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SYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
Decarboxylase, uroporphyrinogen
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-
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Porphyrinogen carboxy-lyase
rl-UroD
UPD
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URO-D
UroD
uroporphyrinogen decarboxylase
Uroporphyrinogen III decarboxylase
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uroporphyrinogen-III decarboxylase
Porphyrinogen carboxy-lyase
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URO-D
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REACTION
REACTION DIAGRAM
COMMENTARY
ORGANISM
UNIPROT
LITERATURE
uroporphyrinogen III = coproporphyrinogen III + 4 CO2
mechanism and stereochemistry
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uroporphyrinogen III = coproporphyrinogen III + 4 CO2
the decarboxylation of uroporphyrinogen occurs in two stages, via 7 COOH and then 6 and 5 COOH intermediates, with the decarboxylation of the 7 COOH compound being the rate-limiting step for the reaction
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uroporphyrinogen III = coproporphyrinogen III + 4 CO2
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-
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REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
decarboxylation
decarboxylation
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-
-
-
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PATHWAY SOURCE
PATHWAYS
BRENDA
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SYSTEMATIC NAME
IUBMB Comments
uroporphyrinogen-III carboxy-lyase (coproporphyrinogen-III-forming)
Acts on a number of porphyrinogens.
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CAS REGISTRY NUMBER
COMMENTARY
9024-70-8
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SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
heptaporphyrinogen III
hexaporphyrinogen III + CO2
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formation of the stepwise decarboxylation products, hepta-, hexa-, penta-, and coproporphyrinogen III
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-
?
hexaporphyrinogen III
pentaporphyrinogen III + CO2
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formation of the stepwise decarboxylation products, hepta-, hexa-, penta-, and coproporphyrinogen III
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-
?
uroporphyrinogen III
heptaporphyrinogen III + CO2
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formation of the stepwise decarboxylation products, hepta-, hexa-, penta-, and coproporphyrinogen III
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-
?
8-carboxylporphyrinogen
coproporphyrinogen(4-COOH) + CO2
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-
-
?
uroporphyrinogen I
coproporphyrinogen I + 4 CO2
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-
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?
uroporphyrinogen I
coproporphyrinogen I + 4 CO2
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formation of the stepwise decarboxylation products, hepta-, hexa-, penta-, and coproporphyrinogen I
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-
?
uroporphyrinogen I
coproporphyrinogen I + 4 CO2
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at 50% of the activity with uroporphyrinogen III
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?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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-
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-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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fifth enzyme of the heme biosynthetic pathway
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?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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fifth step in heme biosynthetic pathway, four sequential decarboxylation reactions
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?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
tetrapyrrole coordination, fifth step in the heme biosynthetic pathway
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?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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fifth enzyme of the heme biosynthetic pathway
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?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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involved in biosynthesis of chlorophyll and haem
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?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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tetrapyrrole biosynthetic pathway, normally associated with pathogen defense
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?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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fifth enzyme of the haem biosynthetic pathway
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?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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heme pathway enzyme, catalyzes the stepwise decarboxylation of porphyrinogen acetic chains
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?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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the enzyme functions in the heme biosynthetic pathway, catalyzing the decarboxylation of the four acetate groups of uroporphyrinogen to form coproporphyrinogen
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-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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with the enzyme from normal animals, uroporphyrinogen and pentacarboxy-porphyrinogen react at the same active site, i.e. the first and fourth decarboxylation occur at the same site. In animals with hexachlorobenzene-induced porphyria the two reactions occur at functionally different sites, but with cross-inhibition. This suggests that hexachlorobenzene causes the two active sites to become nonequivalent and functionally different
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-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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fifth enzyme of the haem biosynthetic pathway
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?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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the decarboxylation of the 7-COOH porphyrinogen is the rate-limiting step
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-
?
uroporphyrinogen-III
coproporphyrinogen-III + 4 CO2
use of Pd/C and hydrogen in reducing uroporphyrin to uroporphyrinogen for use as a substrate for uroporphyrinogen decarboxylase activity assay
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-
?
uroporphyrinogen-III
coproporphyrinogen-III + CO2
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first branching point of the ubiquitous tetrapyrrole biosynthetic pathway, chlorophyll and heme biosynthesis
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?
additional information
?
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the enzyme catalyzes the fifth step in the heme biosynthetic pathway
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?
additional information
?
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porphyria cutanea tarda and hepatoerythropoietic porphyria result from deficiency of uroporphyrinogen decarboxylase
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?
additional information
?
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the familial porphyria cutanea tarda is a genetically determined kinetic abnormality of uroporphyrinogen decarboxylase
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?
additional information
?
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the enzyme is involved in biosynthesis of porphyrin
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?
additional information
?
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porphyria cutanea tarda results from a decreased activity of uroporphyrinogen decarboxylase, the fifth enzyme in heme biosynthesis. The frequency of familial porphyria cutanea tarda in Chile is approximately 50%
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?
additional information
?
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loss of the enzyme activity is a key event in both the induction and the persistence of porphyria
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?
additional information
additional information
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the pattern of accumulation of intermediate 7-COOH-porphyrinogens, 6-COOH-porphyrinogens and 5-COOH-porphyrinogens and final coproporphyrinogen decarboxylation products is affected by the ratio of substrate concentration of uroporphyrinogen I or II to enzyme concentration. Under physiological conditions, where the uroporphyrinogen to enzyme ratio is low, the substrate is nearly quantitatively decarboxylated, and the major product is coproporphyrinogen
?
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NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
uroporphyrinogen-III
coproporphyrinogen-III + CO2
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first branching point of the ubiquitous tetrapyrrole biosynthetic pathway, chlorophyll and heme biosynthesis
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
-
fifth enzyme of the heme biosynthetic pathway
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
-
fifth step in heme biosynthetic pathway, four sequential decarboxylation reactions
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
tetrapyrrole coordination, fifth step in the heme biosynthetic pathway
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
-
fifth enzyme of the heme biosynthetic pathway
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
-
involved in biosynthesis of chlorophyll and haem
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
-
tetrapyrrole biosynthetic pathway, normally associated with pathogen defense
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
-
fifth enzyme of the haem biosynthetic pathway
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
-
heme pathway enzyme, catalyzes the stepwise decarboxylation of porphyrinogen acetic chains
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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the enzyme functions in the heme biosynthetic pathway, catalyzing the decarboxylation of the four acetate groups of uroporphyrinogen to form coproporphyrinogen
-
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
-
fifth enzyme of the haem biosynthetic pathway
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?
additional information
?
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the enzyme catalyzes the fifth step in the heme biosynthetic pathway
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-
?
additional information
?
-
-
porphyria cutanea tarda and hepatoerythropoietic porphyria result from deficiency of uroporphyrinogen decarboxylase
-
-
?
additional information
?
-
-
the familial porphyria cutanea tarda is a genetically determined kinetic abnormality of uroporphyrinogen decarboxylase
-
-
?
additional information
?
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-
the enzyme is involved in biosynthesis of porphyrin
-
-
?
additional information
?
-
-
porphyria cutanea tarda results from a decreased activity of uroporphyrinogen decarboxylase, the fifth enzyme in heme biosynthesis. The frequency of familial porphyria cutanea tarda in Chile is approximately 50%
-
-
?
additional information
?
-
-
loss of the enzyme activity is a key event in both the induction and the persistence of porphyria
-
-
?
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COFACTOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
additional information
enzyme does not require cofactors or prosthetic groups for activity
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additional information
-
enzyme does not require cofactors or prosthetic groups for activity
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METALS and IONS
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
additional information
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INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
7-COOH-porphyrin III
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most effective inhibitor of decarboxylation of both uroporphyrinogen III and 7-COOH-porphyrinogen III