BUP1; Beta-ureidopropionase; BUP-1; Beta-alanine synthase; N-carbamoyl-beta-alanine amidohydrolase

For Research Use Only. Not for use in diagnostic procedures.

E Coli

Greater than 95% as determined by reducing SDS-PAGE.

Supplied as a 0.2 muM filtered solution of 20mM PB, 150mM NaCl, pH 7.4.

Lyophilized protein should be stored at -20 degree C, though stable at room temperature for 3 weeks. Reconstituted protein solution can be stored at 2-8 degree C for 2-7 days. Aliquots of reconstituted samples are stable at -20 degree C for 3 months.

Small volumes of UPB1 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Recombinant Human UPB1 Protein 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.

ELISA (EIA),Western Blot (WB), SDS-PAGE, Mass Spectrometry (MS)

43,166 Da

beta-ureidopropionase 1

beta-ureidopropionase

Beta-ureidopropionase

BUP1??[Similar Products]

BUP1_HUMAN

This gene encodes a protein that belongs to the CN hydrolase family. Beta-ureidopropionase catalyzes the last step in the pyrimidine degradation pathway. 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. UP deficiencies are associated with N-carbamyl-beta-amino aciduria and may lead to abnormalities in neurological activity. [provided by RefSeq, Jul 2008]

UPB1: Converts N-carbamyl-beta-aminoisobutyric acid and N- carbamyl-beta-alanine to, respectively, beta-aminoisobutyric acid and beta-alanine, ammonia and carbon dioxide. Defects in UPB1 are the cause of beta-ureidopropionase deficiency (BUPD). It is characterized by muscular hypotonia, dystonic movements, scoliosis, microcephaly and severe developmental delay. Patients show strongly elevated levels of N- carbamyl-beta-alanine and N-carbamyl-beta-aminoisobutyric acid in plasma, cerebrospinal fluid and urine. Belongs to the CN hydrolase family. BUP subfamily.

Protein type: Cofactor and Vitamin Metabolism - pantothenate and CoA biosynthesis; EC 3.5.1.6; Hydrolase; Nucleotide Metabolism - pyrimidine; Other Amino Acids Metabolism - beta-alanine; Xenobiotic Metabolism - drug metabolism - other enzymes

Chromosomal Location of Human Ortholog: 22q11.2

Cellular Component: cytosol

Molecular Function: beta-ureidopropionase activity

Biological Process: pyrimidine nucleoside catabolic process

Disease: Beta-ureidopropionase Deficiency

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