Uroporphyrinogen III synthase

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Uroporphyrinogen III synthase
Uroporphyrinogen III synthase
Uroporphyrinogen III synthase monomer, Thermus thermophilus

Existing structural data : 1jr2

Properties of human protein
Mass / length primary structure 265 amino acids
Secondary to quaternary structure Monomer
Identifier
Gene name UROS
External IDs
Enzyme classification
EC, category 4.2.1.75 ,  lyase
Response type Ring closure, rearrangement
Substrate Hydroxymethylbilane
Products Uroporphyrinogen III + H 2 O
Occurrence
Parent taxon Creature
Orthologue
human House mouse
Entrez 7390 22276
Ensemble ENSG00000188690; ENSMUSG00000030979
UniProt P10746 P51163
Refseq (mRNA) NM_000375 NM_001302085
Refseq (protein) NP_000366 NP_001289014
Gene locus Chr 10: 125.78 - 125.82 Mb Chr 7: 133.69 - 133.71 Mb
PubMed search 7390 22276

The uroporphyrinogen III synthase (URO3S) is the enzyme in animals that the ring closure of hydroxymethylbilane to Uroporphyrinogen III catalyzes a step in the part Porphyrinbiosynthese . URO3S is found in all tissue types in humans. Mutations in the UROS - gene can URO3S deficiency and this to Günther's disease lead.

Catalyzed reaction

Hydroxymethylbilane ⇒ Uroporphyrinogen III

Hydroxymethylbilane cyclizes to uroporphyrinogen III.

Individual evidence

  1. ↑ Homologues at OMA
  2. ↑ UniProt P10746

Web links

Wikibooks: Biochemistry and Pathobiochemistry: Porphyrin Biosynthesis  - Learning and Teaching Materials