Uroporphyrinogen III synthase

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uroporphyrinogen III synthase
Identifiers
Symbol	UROS
Entrez	7390
HUGO	12592
OMIM	606938
RefSeq	NM_000375
UniProt	P10746
Other data
EC number	4.2.1.75
Locus	Chr. 10 q25.2-26.3

Uroporphyrinogen III synthase is an enzyme involved in the fourth step of porphyrin metabolism, involved in the conversion of hydroxymethyl bilane into uroporphyrinogen III.

Heme synthesis—note that some reactions occur in the cytoplasm and some in the mitochondrion (yellow)

Pathology

A deficiency is associated with Gunther's disease.

v • d • e Metabolism: amino acid metabolism - porphyrin enzymes
Porphyrin biosynthesis	initial mitochondrial: Aminolevulinic acid synthase - Porphobilinogen synthase cytosolic: Porphobilinogen deaminase - Uroporphyrinogen III synthase - Uroporphyrinogen III decarboxylase - Coproporphyrinogen III oxidase terminal mitochondrial: Protoporphyrinogen oxidase - Ferrochelatase
Heme breakdown to bile	spleen: Heme oxygenase - Biliverdin reductase liver: UDP-glucuronosyltransferase
see also disorders, intermediates

v • d • e Carbon-oxygen lyases (EC 4.2) (primarily dehydratases)
Carbonic anhydrase - Fumarase - Aconitase - Enolase (Alpha) - Enoyl-CoA hydratase/3-Hydroxyacyl ACP dehydrase - Methylglutaconyl-CoA hydratase - Tryptophan synthase - Cystathionine beta synthase - Porphobilinogen synthase - 3-isopropylmalate dehydratase - Urocanate hydratase - Uroporphyrinogen III synthase - Nitrile hydratase