Uroporphyrinogen III synthase

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uroporphyrinogen III synthase
Symbol UROS
Entrez 7390
HUGO 12592
OMIM 606938
RefSeq NM_000375
UniProt P10746
Other data
EC number
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)


A deficiency is associated with Gunther's disease.

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