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.
パッケージサイズ | 在庫状況 | 単価(税別) |
---|---|---|
10 μg | 約5 days | ¥ 42,500 |
50 μg | 約5 days | ¥ 125,000 |
500 μg | 約5 days | ¥ 436,000 |
1 mg | 約5 days | ¥ 626,500 |
説明 | 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 Host | E. coli |
Tag | C-6xHis |
Accession Number | Q9UBR1 |
別名 | 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 | Met1-Glu384 |
Protein Purity | Greater than 95% as determined by reducing SDS-PAGE. (QC verified) |
分子量 | 42 KDa (reducing condition) |
Endotoxin | < 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 |
Lyophilized powders can be stably stored for over 12 months, while liquid products can be stored for 6-12 months at-80℃. For reconstituted proteinsolutions, the solution can be stored at -20°c to -80'c for at least 3 months. Please avoid multiple freeze-thaw cycles and store products in aliquots. |
Shipping |
In general, Lyophilized powders are shipping with blue ice. Solutions are shipping with dry ice. |
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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Please read the User Guide of Recombinant Proteins for more specific information.
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