This gene encodes a protein that belongs to the CN hydrolase family. Beta-ureidopropionase catalyzes the last step in the pyrimidine degradation pathway. The pyrimidine bases uracil and thymine are degraded via the consecutive action of dihydropyrimidine dehydrogenase (DHPDH), dihydropyrimidinase (DHP) and beta-ureidopropionase (UP) to beta-alanine and beta-aminoisobutyric acid, respectively. UP deficiencies are associated with N-carbamyl-beta-amino aciduria and may lead to abnormalities in neurological activity. [provided by RefSeq, Jul 2008].
Hu, Lausted, Yoo, Yan, Brightman, Chen, Wang, Bu, Hood: "Quantitative liver-specific protein fingerprint in blood: a signature for hepatotoxicity." in: Theranostics, Vol. 4, Issue 2, pp. 215-28, (2014) (PubMed).