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.
Pack Size | Availability | Price/USD | Quantity |
---|---|---|---|
10 μg | 5 days | $ 184.00 | |
50 μg | 5 days | $ 545.00 | |
500 μg | 5 days | $ 1,900.00 | |
1 mg | 5 days | $ 2,730.00 |
Description | 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. |
Species | Human |
Expression System | E. coli |
Tag | C-6His |
Accession Number | Q9UBR1 |
Synonyms | β-Ureidopropionase, Beta-Alanine Synthase, β-Alanine Synthase, BUP1, UPB1, N-Carbamoyl-β-Alanine Amidohydrolase, Beta-Ureidopropionase, N-Carbamoyl-Beta-Alanine Amidohydrolase, BUP-1 |
Amino Acid | Met1-Glu384 |
Construction | Recombinant Human Beta-Ureidopropionase is produced by our E.coli expression system and the target gene encoding Met1-Glu384 is expressed with a 6His tag at the C-terminus. |
Protein Purity | Greater than 95% as determined by reducing SDS-PAGE. (QC verified) |
Molecular Weight | 42 KDa, reducing conditions |
Endotoxin | Less than 0.1 ng/µg (1 EU/µg) as determined by LAL test. |
Formulation | Supplied as a 0.2 μm filtered solution of PBS, pH 7.4. |
Stability & Storage |
Store at ≤-70°C, stable for 6 months after receipt. Store at ≤-70°C, stable for 3 months under sterile conditions after opening. Please minimize freeze-thaw cycles. |
Shipping |
The product is shipped on dry ice/polar packs. Upon receipt, store it immediately at the temperature listed below. |
Research 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. |
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UPB1 Protein, Human, Recombinant (His) BUP 1 β-Ureidopropionase Beta-Alanine Synthase UPB-1 b-Alanine Synthase β-Alanine Synthase N-Carbamoyl-b-Alanine Amidohydrolase b-Ureidopropionase BUP1 UPB1 N-Carbamoyl-β-Alanine Amidohydrolase Beta-Ureidopropionase N-Carbamoyl-Beta-Alanine Amidohydrolase BUP-1 UPB 1 recombinant recombinant-proteins proteins protein