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uroporphyrinogen-III synthase; UROIIIS; UROS; hydroxymethylbilane hydrolyase [cyclizing]; uroporphyrinogen-III cosynthase (UROS)
Function:
- catalyzes cyclization of the linear tetrapyrrole, hydroxymethylbilane, to the macrocyclic uroporphyrinogen III, the branch point for various sub-pathways leading to a diversity of porphyrins
- porphyrins act as cofactors for many enzymes including methionine synthesis (vitamin B12) or oxygen transport (heme)
- porphyrin metabolism; protoporphyrin-IX biosynthesis
- coproporphyrinogen-III from 5-aminolevulinate: step 3/4
hydroxymethylbilane = uroporphyrinogen III + H2O
Structure:
- monomer
- belongs to the uroporphyrinogen-III synthase family
Expression: ubiquitous
Pathology:
- defects in UROS are the cause of congenital erythropoietic porphyria
Interactions
molecular events
General
hydro-lyase, hydratase or dehydratase
Properties
SIZE: entity length = 265 aa
MW = 29 kD
MOTIF: active site
Database Correlations
OMIM correlations
MORBIDMAP 606938
UniProt P10746
Pfam PF02602
Entrez Gene 7390
Kegg hsa:7390
ENZYME 4.2.1.75
References
- UniProt :accession P10746
- GeneReviews
http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/UROS
- Textbook of Biochemistry with Clinical Correlations,
3rd ed., TM Devlin (ed), Wiley-Liss, NY 1992 pg 1013