UROS transforms HMB to URO3

Stable Identifier
R-HSA-189488
Type
Reaction [transition]
Species
Homo sapiens
Compartment
cytosol
Synonyms
hydroxymethylbilane => H2O + uroporphyrinogen III, Conversion of HMB to uroporphyrinogen III
ReviewStatus
5/5
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UROS transforms HMB to URO3
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Cytosolic uroporphyrinogen III synthase (URO3S) catalyzes the conversion of HMB (hydroxymethylbilane) to uroporphyrinogen III, a reaction involving ring closure and intramolecular rearrangement. Uroporphyrinogen III represents a branch point for the pathways leading to formation of heme, chlorophyll and corrins. HMB is rapidly converted from a linear tetrapyrrole to the cyclic form. Deficiencies of URO3S in vivo are associated with congenital erythropoietic porphyria.
Literature References
PubMed ID Title Journal Year
3805019 Purification and properties of uroporphyrinogen III synthase from human erythrocytes

Tsai, SF, Bishop, DF, Desnick, RJ

J Biol Chem 1987
7592565 Porphobilinogen deaminase and uroporphyrinogen III synthase: structure, molecular biology, and mechanism

Shoolingin-Jordan, PM

J Bioenerg Biomembr 1995
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as an event of
Catalyst Activity

uroporphyrinogen-III synthase activity of UROS [cytosol]

Physical Entity
UROS [cytosol] (Homo sapiens)
Activity
uroporphyrinogen-III synthase activity (GO:0004852)
Orthologous Events
hydroxymethylbilane => uroporphyrinogen III + H2O (Gallus gallus)
UROS transforms HMB to URO3 (Bos taurus)
UROS transforms HMB to URO3 (Canis familiaris)
UROS transforms HMB to URO3 (Danio rerio)
UROS transforms HMB to URO3 (Dictyostelium discoideum)
UROS transforms HMB to URO3 (Drosophila melanogaster)
UROS transforms HMB to URO3 (Gallus gallus)
UROS transforms HMB to URO3 (Mus musculus)
UROS transforms HMB to URO3 (Rattus norvegicus)
UROS transforms HMB to URO3 (Saccharomyces cerevisiae)
UROS transforms HMB to URO3 (Schizosaccharomyces pombe)
UROS transforms HMB to URO3 (Sus scrofa)
UROS transforms HMB to URO3 (Xenopus tropicalis)
Cross References
Rhea
Authored
Jassal, B  (2007-01-24)
D'Eustachio, P  (2007-01-24)
Reviewed
Sassa, S  (2007-01-24)
Created
Jassal, B  (2006-11-20)
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