Product Name
Ureidopropionase Beta (UPb1), Polyclonal Antibody
Full Product Name
Ureidopropionase Beta (UPb1) Polyclonal Antibody
Product Synonym Names
UP-B1; BUP1; Beta-alanine synthase; N-carbamoyl-beta-alanine amidohydrolase
Product Gene Name
anti-UPb1 antibody
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q9UBR1
Species Reactivity
Human, Rat, Pig
Specificity
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.
Purity/Purification
Affinity Chromatography
Form/Format
Supplied as solution form in PBS, pH7.4 containing 0.02% NaN3, 50% glycerol.
Concentration
200ug/ml (lot specific)
Fragment
UPb1 (Trp121~Glu384)
Quality Control
Content: The quality control contains recombinant UPb1 (Trp121~Glu384) disposed in loading buffer.
Usage: 10uL per well when 3,3'-Diaminobenzidine(DAB) as the substrate.
5uL per well when used in enhanced chemilumescent (ECL).
Note: The quality control is specifically manufactured as the positive control.Not used for other purposes.
Loading Buffer: 100mM Tris(pH8.8), 2% SDS, 200mM NaCl, 50% glycerol,BPB 0.01%, NaN3 0.02%.
Conjugated Antibody
The APC conjugated antibody version of this item is also available as catalog #MBS2066901
Preparation and Storage
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.
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 customera??s 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 (EIA)
Application Notes for anti-UPb1 antibody
Western Blot: 1:50-400
Immunohistochemistry in formalin fixed frozen section: 1:50-500
Enzyme-linked Immunosorbent Assay: 1:100-200
Western Blot (WB) of anti-UPb1 antibody
Western Blot: Sample: Lane1: Porcine Liver Tissue; Lane2: Rat Liver Tissue.
Western Blot (WB) of anti-UPb1 antibody
Western Blot: Sample: Recombinant UPb1, Human.
Immunohistochemistry (IHC) of anti-UPb1 antibody
DAB staining on IHC-P; Samples: Human Liver Tissue.
Immunohistochemistry (IHC) of anti-UPb1 antibody
DAB staining on IHC-P; Samples: Human Kidney Tissue
Immunohistochemistry (IHC) of anti-UPb1 antibody
DAB staining on IHC-P; Samples: Human Liver Cancer Tissue.
NCBI/Uniprot data below describe general gene information for UPb1. It may not necessarily be applicable to this product.
NCBI Accession #
NP_057411.1
[Other Products]
NCBI GenBank Nucleotide #
NM_016327.2
[Other Products]
UniProt Primary Accession #
Q9UBR1
[Other Products]
UniProt Secondary Accession #
Q9UIR3; A3KMF8[Other Products]
UniProt Related Accession #
Q9UBR1[Other Products]
Molecular Weight
43,166 Da
NCBI Official Full Name
beta-ureidopropionase
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; metal ion binding
Biological Process: beta-alanine biosynthetic 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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