ureidopropionase, beta
Symbol
UPB1
(may also be known as: None)
Organism
Human
Description
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]
Orthologs
| Symbol | Species | |
|---|---|---|
| upb-1 | Caenorhabditis elegans | |
| pyd3 | Drosophila melanogaster | |
| upb1 | Danio rerio | |
| Upb1 | Mus musculus | |
| Upb1 | Rattus norvegicus |
Links to external resources
Changes associated with this gene
GO Terms
| GO ID | GO Term | GO Category | |
|---|---|---|---|
| GO:0006206 | pyrimidine base metabolic process | biological_process | |
| GO:0006807 | nitrogen compound metabolic process | biological_process | |
| GO:0044281 | small molecule metabolic process | biological_process | |
| GO:0046135 | pyrimidine nucleoside catabolic process | biological_process | |
| GO:0055086 | nucleobase-containing small molecule metabolic process | biological_process | |
| GO:0005737 | cytoplasm | cellular_component | |
| GO:0005829 | cytosol | cellular_component | |
| GO:0003837 | beta-ureidopropionase activity | molecular_function | |
| GO:0016810 | hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds | molecular_function | |
| GO:0046872 | metal ion binding | molecular_function | |