beta-ureidoisobutyrate + H2O => 3-aminoisobutyrate + NH4+ + CO2

Stable Identifier
R-HSA-73620
Type
Reaction
Species
Homo sapiens
Compartment
Synonyms
conversion of 3-Ureidoiosbutyrate to 3-Aminoisobutyrate
Locations in the PathwayBrowser
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Cytosolic UPB1 (beta-ureidopropionase) catalyzes the reaction of 3-ureidoisobutyrate and H2O to form (R)-3-aminoisobutyrate, CO2, and NH3 (Tamaki et al. 2000; van Kuilenburg et al. 2004).

Literature References
PubMed ID Title Journal Year
10989446 Purification, properties, and sequencing of aminoisobutyrate aminotransferases from rat liver

Matsuda, K, Sakata, SF, Tamaki, N

Methods Enzymol 2000
15385443 beta-Ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities

van Lenthe, H, van Gennip, AH, Zoetekouw, L, Ribes, A, van Kuilenberg, AB, Beke, E, van Cruchten, A, Meinsma, R, Hoffmann, GF, Wevers, RA, Kulik, W, Busch, R, Mayatepek, E, Rutsch, F, Voit, T, Assmann, B, Abeling, NG, Lorente, I, Stroomer, AE

Hum Mol Genet 2004
Participants
Participates
Catalyst Activity

beta-ureidopropionase activity of UPB1:Zn2+ [cytosol]

Orthologous Events
Cross References
Rhea
Authored
Created
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