Recombinant Human BUP1 Protein (His Tag)

C-His

Store at < -20°C, stable for 6 months. Please minimize freeze-thaw cycles.

42 kDa

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.

< 1.0 EU per μg of the protein as determined by the LAL method.

Supplied as a 0.2 μm filtered solution of PBS, pH7.4.

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.