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

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

Stable Identifier

R-HSA-73620

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

conversion of 3-Ureidoiosbutyrate to 3-Aminoisobutyrate

ReviewStatus

5/5

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

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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