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

Stable Identifier
R-HSA-73620
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
conversion of 3-Ureidoiosbutyrate to 3-Aminoisobutyrate
Locations in the PathwayBrowser
General
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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
15385443 beta-Ureidopropionase deficiency: an inborn error of pyrimidine degradation associated with neurological abnormalities

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

Hum Mol Genet 2004
10989446 Purification, properties, and sequencing of aminoisobutyrate aminotransferases from rat liver

Tamaki, N, Sakata, SF, Matsuda, K

Methods Enzymol 2000
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
beta-ureidopropionase activity of UPB1:Zn2+ [cytosol]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
Authored
Created