Name: Human BUP1 Recombinant Protein (His Tag)
Synonyms: Beta-Ureidopropionase;BUP-1;Beta-Alanine Synthase;N-Carbamoyl-Beta-Alanine Amidohydrolase;UPB1;BUP1
Expression host: E.coli
Sequence: Met 1-Glu384
Accesstion: Q9UBR1
Species: Human
Mol_Mass: 44.2 kDa
AP_Mol_Mass: 42 kDa
Tag: C-His
Purity: > 95 % as determined by reducing SDS-PAGE.
Endotoxin:
Storage: Store at
Shipping: This product is provided as liquid. It is shipped at frozen temperature with blue ice/gel packs. Upon receipt, store it immediately at
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.
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