Guinea Pig Beta-ureidopropionase (UPB1) ELISA Kit | UPB1 elisa kit
Guinea pig Beta-ureidopropionase (UPB1) ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
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 Description
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: Xenobiotic Metabolism - drug metabolism - other enzymes; Hydrolase; Nucleotide Metabolism - pyrimidine; EC 3.5.1.6; Cofactor and Vitamin Metabolism - pantothenate and CoA biosynthesis; Other Amino Acids Metabolism - beta-alanine
Chromosomal Location of Human Ortholog: 22q11.2
Cellular Component: cytosol
Molecular Function: metal ion binding; beta-ureidopropionase activity
Biological Process: pyrimidine nucleoside catabolic process; pyrimidine base metabolic process; nucleobase, nucleoside and nucleotide metabolic process; beta-alanine biosynthetic process
Disease: Beta-ureidopropionase Deficiency
Research Articles on UPB1
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Product Notes
The Guinea Pig UPB1 upb1 (Catalog #AAA7219521) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA7219521 ELISA Kit recognizes Guinea Pig UPB1. It is sometimes possible for the material contained within the vial of "Beta-ureidopropionase (UPB1), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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