Mouse Anti-UPB1 Recombinant Antibody (3F12) (CBMAB-C4690-CN)

Name: Mouse Anti-UPB1 Recombinant Antibody (3F12)
SKU: CBMAB-C4690-CN
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Mouse

Clone

3F12

Application

ELISA, IHC, WB

Immunogen

Partial recombinant corresponding to aa 285-385 from human UPB1 (NP_057411) with GST tag

Specificity

Human

Antibody Isotype

IgG1, κ

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!]

Format

Liquid

Buffer

PBS, pH 7.2

More Infomation

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

51733

UniProt ID

Q9UBR1

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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

Isotype control

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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