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Product Name | Ureidopropionase, beta (UPB1) (AA 1-384) protein (His tag) |
Description | beta-Ureidopropionase is a cytoplasmic protein which belongs to the CN hydrolase family of BUP subfamily. beta-Ureidopropionase binds one zinc ion per subunit, catalyzes the last step in the pyrimidine degradation pathway. beta-Ureidopropionase can convert N-carbamyl-beta-aminoisobutyric acid and N-carbamyl-beta-alanine to beta-aminoisobutyric acid and beta-alanine, ammonia and carbon dioxide, respectively. 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. Defects in beta-Ureidopropionase are the cause of beta-Ureidopropionase deficiency that is characterized by muscular hypotonia, dystonic movements, scoliosis, microcephaly and severe developmental delay.Alternative Names: Beta-Ureidopropionase, BUP-1, Beta-Alanine Synthase, N-Carbamoyl-Beta-Alanine Amidohydrolase, UPB1, BUP1 |
Size | 500 µg |
Concentration | n/a |
Applications | n/a |
Other Names | MGC82230|wu:fb69e03|zgc:64020|BUP1|AI195023|Bup1 |
Gene, Accession, CAS # | Gene ID: 51733 |
Catalog # | ABIN1690951 |
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Order / More Info | Ureidopropionase, beta (UPB1) (AA 1-384) protein (His tag) from ANTIBODIES-ONLINE GmbH |
Product Specific References | n/a |
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