UROS transforms HMB to URO3

Stable Identifier
R-HSA-189488
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
Conversion of HMB to uroporphyrinogen III
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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
7592565 Porphobilinogen deaminase and uroporphyrinogen III synthase: structure, molecular biology, and mechanism

Shoolingin-Jordan, PM

J Bioenerg Biomembr 1995
3805019 Purification and properties of uroporphyrinogen III synthase from human erythrocytes

Tsai, SF, Bishop, DF, Desnick, RJ

J Biol Chem 1987
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
uroporphyrinogen-III synthase activity of UROS [cytosol]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
Authored
Reviewed
Created