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
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4.2.1.75
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porphyria
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erythropoietic
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heme
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photosensitivity
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5-aminolevulinic
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tetrapyrrole
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coproporphyrins
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delta-aminolevulinic
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ferrochelatase
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pbgase
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mutilate
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erythrodontia
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transfusion-dependent
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protoporphyria
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hypertrichosis
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urologists
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coproporphyrinogen
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aymaras
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amerindian
- 4.2.1.75
- porphyria
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erythropoietic
- heme
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photosensitivity
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5-aminolevulinic
- tetrapyrrole
- coproporphyrins
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delta-aminolevulinic
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ferrochelatase
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pbgase
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mutilate
-
erythrodontia
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transfusion-dependent
- protoporphyria
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hypertrichosis
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urologists
- coproporphyrinogen
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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
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Natural Substrates Products
Natural Substrates Products on EC 4.2.1.75 - uroporphyrinogen-III synthase
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REACTION DIAGRAM
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synthesis of uroporphyrinogen III, key intermediate for biosynthesis of tetrapyrrolic compounds like chlorophylls, hemes, cytochromes and vitamin B12
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Hydroxymethylbilane
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synthesis of uroporphyrinogen III, key intermediate for biosynthesis of tetrapyrrolic compounds like chlorophylls, hemes, cytochromes and vitamin B12
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Hydroxymethylbilane
Uroporphyrinogen III
Thermus thermophilus HB27 / ATCC BAA-163 / DSM 7039
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uroporphyrinogen III + H2O
a step in tetrapyrrole biosynthesis, e.g. of chlorophyll, haem, sirohaem and bilins, overview
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hydroxymethylbilane
uroporphyrinogen III + H2O
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intramolecular rearrangement of the d-pyrrole group and ring closure
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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
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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
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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
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hydroxymethylbilane
uroporphyrinogen-III + H2O
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macrocyclic, role of the enzyme in tetrapyrrole based copound biosynthesis, overview
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hydroxymethylbilane
uroporphyrinogen-III + H2O
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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
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uroporphyrinogen III synthase functions as heme synthesis enzyme during hematopoietic development of Danio rerio
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additional information
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uroporphyrinogen III synthase functions as heme synthesis enzyme during hematopoietic development of Danio rerio
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additional information
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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
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