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Recombinant Human BUP1 Protein (His Tag)
SKU: PKSH033271-50
Recombinant Human BUP1 Protein (His Tag)
| SKU # | PKSH033271 |
| Expression Host | E.coli |
Description
| Synonyms | BUP-1, BUP1, Beta-Alanine Synthase, Beta-Ureidopropionase, N-Carbamoyl-Beta-Alanine Amidohydrolase, UPB1 |
| Species | Human |
| Expression Host | E.coli |
| Sequence | Met 1-Glu384 |
| Accession | Q9UBR1 |
| Calculated Molecular Weight | 44.2 kDa |
| Observed Molecular Weight | 42 kDa |
| Tag | C-His |
| Bio-activity | Not validated for activity |
Properties
| Purity | > 95 % as determined by reducing SDS-PAGE. |
| Endotoxin | < 1.0 EU per μg of the protein as determined by the LAL method. |
| Storage | Store at < -20°C, stable for 6 months. Please minimize freeze-thaw cycles. |
| Shipping | This product is provided as liquid. It is shipped at frozen temperature with blue ice/gel packs. Upon receipt, store it immediately at < - 20°C. |
| Formulation | Supplied as a 0.2 μm filtered solution of PBS, pH7.4. |
| Reconstitution | Not Applicable |
Background
β-Ureidopropionase is a cytoplasmic protein which belongs to the CN hydrolase family of BUP subfamily. β-Ureidopropionase binds one zinc ion per subunit, catalyzes the last step in the pyrimidine degradation pathway. β-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 β-Ureidopropionase are the cause of β-Ureidopropionase deficiency that is characterized by muscular hypotonia, dystonic movements, scoliosis, microcephaly and severe developmental delay.