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Information on EC 4.2.1.75 - uroporphyrinogen-III synthase and Organism(s) Mus musculus and UniProt Accession P51163

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EC Tree
     4 Lyases
         4.2 Carbon-oxygen lyases
             4.2.1 Hydro-lyases
                4.2.1.75 uroporphyrinogen-III synthase
IUBMB Comments
In the presence of EC 2.5.1.61, hydroxymethylbilane synthase, the enzyme forms uroporphyrinogen III from porphobilinogen.
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This record set is specific for:
Mus musculus
UNIPROT: P51163
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Word Map
The taxonomic range for the selected organisms is: Mus musculus
The expected taxonomic range for this enzyme is: Bacteria, Eukaryota, Archaea
Synonyms
uros, uroporphyrinogen iii synthase, porphobilinogenase, uro-synthase, uroiiis, uroporphyrinogen iii cosynthase, u3s, uro synthase, uro'gen iii synthase, uroporphyrinogen-iii synthase, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
URO-synthase
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Uroporphyrinogen III synthase
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Hydroxymethylbilane hydrolyase [cyclizing]
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-
-
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Isomerase, uroporphyrinogen
-
-
-
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Porphobilinogenase
-
-
-
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Synthase, uroporphyrinogen III co-
-
-
-
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URO-synthase
-
-
-
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UROIIIS
-
-
-
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Uroporphyrinogen III co-synthase
-
-
-
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Uroporphyrinogen III cosynthase
-
-
-
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Uroporphyrinogen III synthase
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-
-
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Uroporphyrinogen isomerase
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-
-
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Uroporphyrinogen-III cosynthase
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-
-
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Uroporphyrinogen-III cosynthetase
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-
-
-
UROS
-
-
-
-
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
elimination
-
-
-
-
SYSTEMATIC NAME
IUBMB Comments
hydroxymethylbilane hydro-lyase (cyclizing; uroporphyrinogen-III-forming)
In the presence of EC 2.5.1.61, hydroxymethylbilane synthase, the enzyme forms uroporphyrinogen III from porphobilinogen.
CAS REGISTRY NUMBER
COMMENTARY hide
37340-55-9
-
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
Hydroxymethylbilane
Uroporphyrinogen III
show the reaction diagram
-
-
-
?
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
IMAGE
Coproporphyrins
-
-
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uroporphyrinogen III
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product inhibition
Uroporphyrins
-
-
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
7.7 - 7.9
-
-
pH RANGE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
6.5 - 8.5
-
at pH 6.5: 10% of optimum activity, at pH 8.5: 30% of optimum activity
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
-
SwissProt
Manually annotated by BRENDA team
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
-
-
Manually annotated by BRENDA team
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
HEM4_MOUSE
265
0
28504
Swiss-Prot
other Location (Reliability: 2)
PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
C73R
0.24% of wild-type activity, homozygous mice are fetal lethals
V99A
5.9% of wild-type activity, homozygous mice are fetal lethals
V99L
14.8% of wild-type activity
PURIFICATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
expression in Escherichia coli
REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Levin, E.Y.
Enzymatic properties of uroporphyrinogen III cosynthetase
Biochemistry
10
4669-4675
1971
Mus musculus
Manually annotated by BRENDA team
Bishop, D.F.; Johansson, A.; Phelps, R.; Shady, A.A.; Ramirez, M.C.; Yasuda, M.; Caro, A.; Desnick, R.J.
Uroporphyrinogen III synthase knock-in mice have the human congenital erythropoietic porphyria phenotype, including the characteristic light-induced cutaneous lesions
Am. J. Hum. Genet.
78
645-658
2006
Mus musculus (P51163), Mus musculus
Manually annotated by BRENDA team