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]
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Function:
Catalyzes a late step in pyrimidine degradation (PubMed:22525402, PubMed:24526388). Converts N-carbamoyl-beta-alanine (3-ureidopropanoate) into beta-alanine, ammonia and carbon dioxide (PubMed:10415095, PubMed:10542323, PubMed:11508704, PubMed:22525402, PubMed:24526388, PubMed:29976570). Likewise, converts N-carbamoyl-beta-aminoisobutyrate (3-ureidoisobutyrate) into beta-aminoisobutyrate, ammonia and carbon dioxide (Probable). {ECO:0000269|PubMed:10415095, ECO:0000269|PubMed:10542323, ECO:0000269|PubMed:11508704, ECO:0000269|PubMed:22525402, ECO:0000269|PubMed:24526388, ECO:0000269|PubMed:29976570, ECO:0000305|PubMed:22525402, ECO:0000305|PubMed:24526388}.
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Cellular Localization:
Cytoplasm.
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Tissue Expression:
Detected in liver (at protein level). {ECO:0000269|PubMed:22525402}.