Product Name
beta-Ureidopropionase/UPB1, Recombinant Protein
Full Product Name
Recombinant Human beta-Ureidopropionase/UPB1 Protein (C-6His)
Product Synonym Names
Beta-Ureidopropionase; BUP-1; Beta-Alanine Synthase; N-Carbamoyl-Beta-Alanine Amidohydrolase; UPB1; BUP1
Product Gene Name
UPB1 recombinant protein
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence Positions
Met1-Glu384
3D Structure
ModBase 3D Structure for Q9UBR1
Purity/Purification
>90% as determined by reducing SDS-PAGE.
Form/Format
Supplied as a 0.2 mum filtered solution of 20mM PB, 150mM NaCl, pH 7.4.
Endotoxin
<1.0 EU per ug as determined by LAL test.
Preparation and Storage
Store at < -20 degree C, stable for 6 months after receipt. Please minimize freeze-thaw cycles.
ISO Certification
Manufactured in an ISO 9001:2015 Certified Laboratory.
Other Notes
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.
Related Product Information for
UPB1 recombinant protein
beta-Ureidopropionase is a cytoplasmic protein which belongs to the CN hydrolase family of BUP subfamily. beta-Ureidopropionase binds one zinc ion per subunit, catalyzes the last step in the pyrimidine degradation pathway. beta-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 beta-Ureidopropionase are the cause of beta-Ureidopropionase deficiency that is characterized by muscular hypotonia, dystonic movements, scoliosis, microcephaly and severe developmental delay.
NCBI/Uniprot data below describe general gene information for UPB1. It may not necessarily be applicable to this product.
NCBI Accession #
NP_057411.1
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NCBI GenBank Nucleotide #
NM_016327.2
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UniProt Primary Accession #
Q9UBR1
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UniProt Secondary Accession #
Q9UIR3; A3KMF8[Other Products]
UniProt Related Accession #
Q9UBR1[Other Products]
Molecular Weight
Molecular Mass: 44.2 kDa
Actual Protein Molecular Mass: 42 kDa
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
Converts N-carbamoyl-beta-aminoisobutyrate and N-carbamoyl-beta-alanine (3-ureidopropanoate) to, respectively, beta-aminoisobutyrate and beta-alanine, ammonia and carbon dioxide.
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
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.
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