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Rabbit Anti-UPB1 Recombinant Antibody (CBCNC-797) (CBMAB-C4692-CN)

This product is a Rabbit antibody that recognizes UPB1. The antibody CBCNC-797 can be used for immunoassay techniques such as: WB, IP, IHC-P.
See all UPB1 antibodies

Summary

Host Animal
Rabbit
Specificity
Human
Clone
CBCNC-797
Antibody Isotype
IgG
Application
WB, IP, IHC-P

Basic Information

Immunogen
Synthetic peptide within Human UPB1 aa 350-450 (C terminal). The exact sequence is proprietary.
Specificity
Human
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Buffer
Tissue culture supernatant

Target

Full Name
ureidopropionase, beta
Introduction
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]
Entrez Gene ID
UniProt ID
Alternative Names
Beta-Ureidopropionase 1; N-Carbamoyl-Beta-Alanine Amidohydrolase; Ureidopropionase, Beta; Beta-Alanine Synthase; EC 3.5.1.6; BUP1; Beta-Ureidopropionase; BUP-1;
Function
Catalyzes a late step in pyrimidine degradation (PubMed:22525402, PubMed:24526388).
Converts N-carbamoyl-beta-alanine (3-ureidopropanoate) into beta-alanine, ammonia and carbon dioxide (PubMed:10542323, PubMed:11508704, PubMed:10415095, PubMed:29976570, PubMed:22525402, PubMed:24526388).
Likewise, converts N-carbamoyl-beta-aminoisobutyrate (3-ureidoisobutyrate) into beta-aminoisobutyrate, ammonia and carbon dioxide (Probable).
Biological Process
Biological Process beta-alanine biosynthetic process via 3-ureidopropionate Source:UniProtKB2 Publications
Biological Process CMP catabolic process Source:Ensembl
Biological Process dCMP catabolic process Source:Ensembl
Biological Process dUMP catabolic process Source:Ensembl
Biological Process in utero embryonic development Source:Ensembl
Biological Process liver development Source:Ensembl
Biological Process protein homooligomerization Source:UniProtKB1 Publication
Biological Process protein homotetramerization Source:UniProtKB1 Publication
Biological Process pyrimidine nucleoside catabolic process Source:UniProtKB1 Publication
Biological Process UMP catabolic process Source:Ensembl
Cellular Location
Cytoplasm
Involvement in disease
Beta-ureidopropionase deficiency (UPB1D):
An inborn error of metabolism due to a defect in pyrimidine degradation. 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.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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