4.1.1.37: uroporphyrinogen decarboxylase
This is an abbreviated version!
For detailed information about uroporphyrinogen decarboxylase, go to the full flat file.
Word Map on EC 4.1.1.37
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4.1.1.37
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porphyria
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cutanea
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tarda
-
heme
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erythrocyte
-
porphyrinogenic
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hexachlorobenzene
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urinary
-
hepatoerythropoietic
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porphobilinogen
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overload
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photosensitivity
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ferrochelatase
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protoporphyrin
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hemochromatosis
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5-aminolaevulinate
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heptacarboxylic
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hexachlorobenzene-induced
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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
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protoporphyrinogen
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uroporphyria
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tetrapyrrole
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sun-exposed
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blister
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siderosis
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diagnostics
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3,4,3\',4\'-tetrachlorobiphenyl
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medicine
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hcb-induced
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miliae
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coproporphyria
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bulla
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agriculture
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hepta
- 4.1.1.37
- porphyria
-
cutanea
- tarda
- heme
- erythrocyte
-
porphyrinogenic
- hexachlorobenzene
- urinary
-
hepatoerythropoietic
- porphobilinogen
- overload
-
photosensitivity
-
ferrochelatase
- protoporphyrin
- hemochromatosis
-
5-aminolaevulinate
-
heptacarboxylic
-
hexachlorobenzene-induced
- hypertrichosis
-
phlebotomy
-
erythropoietic
-
delta-aminolaevulinic
-
polyhalogenated
-
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
Reaction
Synonyms
ch-UroD, Decarboxylase, uroporphyrinogen, Hem12p, PCL, Porphyrinogen carboxy-lyase, rl-UroD, tobacco UROD, UORO-D, UPD, URO-D, Uro-decarboxylase, uro-III decarboxylase, UroD, UROD protein, UROD1, uroporphyrinogen decarboxylase, uroporphyrinogen decarboxylase 1, Uroporphyrinogen III decarboxylase, uroporphyrinogen-decarboxylase, uroporphyrinogen-III decarboxylase
ECTree
4.1.1.37 uroporphyrinogen decarboxylaseAdvanced search results
results (
results (Substrates Products
Substrates Products on EC 4.1.1.37 - uroporphyrinogen decarboxylase
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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
-
formation of the stepwise decarboxylation products, hepta-, hexa-, penta-, and coproporphyrinogen III
-
-
?
hexaporphyrinogen III
pentaporphyrinogen III + CO2
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formation of the stepwise decarboxylation products, hepta-, hexa-, penta-, and coproporphyrinogen III
-
-
?
uroporphyrinogen III
heptaporphyrinogen III + CO2
-
formation of the stepwise decarboxylation products, hepta-, hexa-, penta-, and coproporphyrinogen III
-
-
?
8-carboxylporphyrinogen
coproporphyrinogen(4-COOH) + CO2
-
-
-
?
uroporphyrinogen I
coproporphyrinogen I + 4 CO2
-
-
-
-
?
uroporphyrinogen I
coproporphyrinogen I + 4 CO2
-
formation of the stepwise decarboxylation products, hepta-, hexa-, penta-, and coproporphyrinogen I
-
-
?
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
-
-
-
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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fifth enzyme of the heme biosynthetic pathway
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
-
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
-
?
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
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?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
-
tetrapyrrole biosynthetic pathway, normally associated with pathogen defense
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?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
-
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
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
-
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
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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
-
fifth enzyme of the haem biosynthetic pathway
-
?
Uroporphyrinogen III
Coproporphyrinogen III + 4 CO2
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the decarboxylation of the 7-COOH porphyrinogen is the rate-limiting step
-
-
?
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
-
first branching point of the ubiquitous tetrapyrrole biosynthetic pathway, chlorophyll and heme biosynthesis
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?
additional information
?
-
-
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
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-
?
additional information
?
-
-
the familial porphyria cutanea tarda is a genetically determined kinetic abnormality of uroporphyrinogen decarboxylase
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-
?
additional information
?
-
-
the enzyme is involved in biosynthesis of porphyrin
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-
?
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%
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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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-
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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
?
