4.2.1.75: uroporphyrinogen-III synthase
This is an abbreviated version!
For detailed information about uroporphyrinogen-III synthase, go to the full flat file.
Word Map on EC 4.2.1.75
-
4.2.1.75
-
porphyria
-
erythropoietic
-
heme
-
photosensitivity
-
5-aminolevulinic
-
tetrapyrrole
-
coproporphyrins
-
delta-aminolevulinic
-
ferrochelatase
-
pbgase
-
mutilate
-
erythrodontia
-
transfusion-dependent
-
protoporphyria
-
hypertrichosis
-
urologists
-
coproporphyrinogen
-
aymaras
-
amerindian
- 4.2.1.75
- porphyria
-
erythropoietic
- heme
-
photosensitivity
-
5-aminolevulinic
- tetrapyrrole
- coproporphyrins
-
delta-aminolevulinic
-
ferrochelatase
-
pbgase
-
mutilate
-
erythrodontia
-
transfusion-dependent
- protoporphyria
-
hypertrichosis
-
urologists
- coproporphyrinogen
-
aymaras
-
amerindian
Reaction
Synonyms
CobA/HemD, Hydroxymethylbilane hydrolyase [cyclizing], hydroxymethylbilane hydrolyase, cyclizing, Isomerase, uroporphyrinogen, Porphobilinogenase, Synthase, uroporphyrinogen III co-, U3S, URO synthase, uro'gen III synthase, URO-synthase, UROIIIS, Uroporphyrinogen III co-synthase, Uroporphyrinogen III cosynthase, Uroporphyrinogen III synthase, Uroporphyrinogen isomerase, Uroporphyrinogen-III cosynthase, Uroporphyrinogen-III cosynthetase, uroporphyrinogen-III-synthase, UROS
ECTree
4.2.1.75 uroporphyrinogen-III synthaseAdvanced search results
results (
results (Substrates Products
Substrates Products on EC 4.2.1.75 - uroporphyrinogen-III synthase
Please wait a moment until all data is loaded. This message will disappear when all data is loaded.
top
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
Hydroxymethylbilane
?
-
synthesis of uroporphyrinogen III, key intermediate for biosynthesis of tetrapyrrolic compounds like chlorophylls, hemes, cytochromes and vitamin B12
-
-
?
Hydroxymethylbilane
?
-
synthesis of uroporphyrinogen III, key intermediate for biosynthesis of tetrapyrrolic compounds like chlorophylls, hemes, cytochromes and vitamin B12
-
-
?
Hydroxymethylbilane
Uroporphyrinogen III
-
enzyme-product structure analysis, overview
-
?
Hydroxymethylbilane
Uroporphyrinogen III
Thermus thermophilus HB27 / ATCC BAA-163 / DSM 7039
-
enzyme-product structure analysis, overview
-
?
hydroxymethylbilane
uroporphyrinogen III + H2O
a step in tetrapyrrole biosynthesis, e.g. of chlorophyll, haem, sirohaem and bilins, overview
-
-
?
hydroxymethylbilane
uroporphyrinogen III + H2O
uroporphyrinogen III synthase catalyses the cyclization of HMB with a concomitant inversion of the fourth ring of the porphyrin macrocycle, giving rise to uroporphyrinogen III
-
-
?
hydroxymethylbilane
uroporphyrinogen III + H2O
-
intramolecular rearrangement of the d-pyrrole group and ring closure
-
-
?
hydroxymethylbilane
uroporphyrinogen III + H2O
the D-ring of the hydroxymethylbilane substrate binds to the enzyme in a conformation that shields its terminal portion from reacting with ring A and prevents the formation of the biologically useless uroporphyrinogen I, reaction mechanism, overview
-
-
?
hydroxymethylbilane
uroporphyrinogen III + H2O
the enzyme catalyzes the cyclization and D-ring isomerization of hydroxymethylbilane to uroporphyrinogen III, the cyclic tetrapyrrole and physiologic precursor of heme, chlorophyl, and corrin
-
-
?
hydroxymethylbilane
uroporphyrinogen III + H2O
the enzyme catalyzes the inversion of one of the four heterocyclic rings present in the substrate. Reaction mechanism analysis by high-level quantum mechanical calculations on model systems of the enzyme
-
-
?
hydroxymethylbilane
uroporphyrinogen III + H2O
-
enzymatic conversion of preuroporphyrinogen. The substrate must bind the enzyme in a conformation that prevents C19 from reacting with C20
-
-
?
hydroxymethylbilane
uroporphyrinogen-III + H2O
linear tetrapyrrole, fourth step in the biosynthesis of porphyrin, essential reaction, decreased enzyme activity leads to the autosomal recessive disorder congenital erythropetic porphyria
macrocyclic uroporphyrinogen III is a precurosr for synthesis of diverse compounds, e.g. heme, siroheme, chlorophyll, F430, and vitamin B12
?
hydroxymethylbilane
uroporphyrinogen-III + H2O
-
macrocyclic, role of the enzyme in tetrapyrrole based copound biosynthesis, overview
-
?
hydroxymethylbilane
uroporphyrinogen-III + H2O
-
fourth enzyme in heme biosynthesis. Congenital erythropoietic porphyria is a very rare disease that is inherited as an autosomal recessive trait and results from a profound deficiency of uroporphyrinogen III cosynthase, the fourth enzyme in heme biosynthesis. The degree of severity of clinical symptoms mainly depends on the amount of residual uroporphyrinogen III cosynthase activity
-
-
?
hydroxymethylbilane
uroporphyrinogen-III + H2O
-
-
-
?
additional information
?
-
coupled assay method for UROS activity measurement using purified recombinant Pseudomonas aeruginosa porphobilinogen synthase and Bacillus megaterium porphobilinogen deaminase to generate hydroxymethylbilane, the substrate for UROS, enzymatically from 5-aminolaevulinic acid
-
-
?
additional information
?
-
-
coupled assay method for UROS activity measurement using purified recombinant Pseudomonas aeruginosa porphobilinogen synthase and Bacillus megaterium porphobilinogen deaminase to generate hydroxymethylbilane, the substrate for UROS, enzymatically from 5-aminolaevulinic acid
-
-
?
additional information
?
-
uroporphyrinogen III synthase functions as heme synthesis enzyme during hematopoietic development of Danio rerio
-
-
?
additional information
?
-
-
uroporphyrinogen III synthase functions as heme synthesis enzyme during hematopoietic development of Danio rerio
-
-
?
additional information
?
-
-
hydroxymethylbilane synthase and uroporphyrinogen III synthase catalyze two consecutive reactions, the third and fourth step, in the heme biosynthetic pathway, generating the first linear and the first cyclic tetrapyrroles, respectively. Hydroxymethylbilane synthase, 2.5.1.61, and uroporphyrinogen III synthase may function independently and sequentially with hydroxymethylbilane as a free intermediate, heme biosynthetic pathway, overview. Hypoxia downregulates UROS mRNA expression in hepatic cells, reduction of UROS mRNA is associated with the accumulation of hypoxia-inducible factor 1alpha under normoxia. Deferoxamine, cobalt chloride, or hypoxia downregulate UROS mRNA expression in hepatic cells, reduction of UROS mRNA is associated with the accumulation of hypoxia-inducible factor 1alpha under normoxia
-
-
?
