UP-B1; BUP1; Beta-alanine synthase; N-carbamoyl-beta-alanine amidohydrolase

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

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against UPb1. It has been selected for its ability to recognize UPb1 in immunohistochemical staining and western blotting.

Affinity Chromatography

Supplied as solution form in PBS, pH7.4 containing 0.02% NaN3, 50% glycerol.

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of anti-UPb1 antibody 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.

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA)

Western Blot: 1:50-400
Immunohistochemistry in formalin fixed frozen section: 1:50-500
Enzyme-linked Immunosorbent Assay: 1:100-200

Western Blot: Sample: Recombinant protein.

DAB staining on fromalin fixed paraffin- embedded Kidney tissue)

43,937 Da

ureidopropionase, beta

beta-ureidopropionase

Beta-ureidopropionase

This gene encodes an enzyme that catalyzes the conversion of N-carbamyl-beta-aminoisobutyric acid and N-carbamyl-beta-alanine to beta-aminoisobutyric acid or beta-alanine. Mutations in the related gene in human have been associated with beta-ureidopropionase deficiency. [provided by RefSeq, Dec 2013]

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: 10|10 C1

Cellular Component: cytoplasm

Molecular Function: beta-ureidopropionase activity; catalytic activity; hydrolase activity; hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds; metal ion binding; zinc ion binding

Biological Process: beta-alanine metabolic process; liver development; metabolic process; nitrogen compound metabolic process

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