Product Name
Ureidopropionase Beta (UPb1), Polyclonal Antibody
Full Product Name
FITC-Linked Polyclonal Antibody to Ureidopropionase Beta (UPb1)
Product Synonym Names
UP-B1; BUP1; Beta-alanine synthase; N-carbamoyl-beta-alanine amidohydrolase
Product Gene Name
anti-UPb1 antibody
[Similar Products]
Matching Pairs
Unconjugated Antibody: Ureidopropionase Beta (MBS2032439)
FITC Conjugated Antibody: Ureidopropionase Beta (UPb1) (MBS2066903)
Matching Pairs
FITC Conjugated Antibody: Ureidopropionase Beta (UPb1) (MBS2066903)
Immunogen: Ureidopropionase Beta (MBS2033031)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Concentration
200ug/ml (lot specific)
Immunogen
UPb1 (Trp121~Glu384)
Unconjugated Antibody
The unconjugated antibody version of this item is also available as catalog #MBS2032439
ISO Certification
Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.
Supply Chain Verification
Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customerâs specifications, please inquire.
Other Notes
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.
Applications Tested/Suitable for anti-UPb1 antibody
Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (ELISA)
NCBI/Uniprot data below describe general gene information for UPb1. It may not necessarily be applicable to this product.
NCBI Accession #
EAW59664.1
[Other Products]
UniProt Secondary Accession #
Q9UIR3; A3KMF8[Other Products]
UniProt Related Accession #
Q9UBR1[Other Products]
Molecular Weight
43,166 Da
NCBI Official Full Name
ureidopropionase, beta, isoform CRA_b
NCBI Official Synonym Full Names
beta-ureidopropionase 1
NCBI Official Symbol
UPB1 [Similar Products]
NCBI Official Synonym Symbols
BUP1
[Similar Products]
NCBI Protein Information
beta-ureidopropionase
UniProt Protein Name
Beta-ureidopropionase
UniProt Synonym Protein Names
BUP-1; Beta-alanine synthase; N-carbamoyl-beta-alanine amidohydrolase
Protein Family
Transcription factor
UniProt Gene Name
UPB1 [Similar Products]
UniProt Synonym Gene Names
BUP1 [Similar Products]
NCBI Summary for UPb1
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]
UniProt Comments for UPb1
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: 22q11.23
Cellular Component: cytosol
Molecular Function: beta-ureidopropionase activity
Biological Process: pyrimidine nucleoside catabolic process
Disease: Beta-ureidopropionase Deficiency
Research Articles on UPb1
1. A (p.R326Q) is the most common mutation of the UPB1 gene in Chinese. The predicted incidence indicates that beta-ureidopropionase deficiency is significantly underdiagnosed in the Chinese population.">The c.977G>A (p.R326Q) is the most common mutation of the UPB1 gene in Chinese. The predicted incidence indicates that beta-ureidopropionase deficiency is significantly underdiagnosed in the Chinese population.
Precautions
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Disclaimer
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