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The antibody against UPB1 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-240 of human UPB1 (NP_057411.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
The antibody against UPB1 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-240 of human UPB1 (NP_057411.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
| Cat.No | ADA-05047A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | UPB1 |
| Target Synonyms | BUP1; UPB1 | Form | Liquid |
| Species Reactivity | Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.01% thimerosal, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | Mouse kidney, Mouse liver, Rat kidney | Application | ELISA, WB |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 1-240 of human UPB1 (NP_057411.1). | Target Species | Human |
|---|---|---|---|
| Uniprot ID | Q9UBR1 | Immunogen Sequence |
Uniprot Id
Q9UBR1
Target Species
Human
Target Name
UPB1
Target Full Name
Beta-ureidopropionase
Target Function
Catalyzes a late step in pyrimidine degradation. Converts N-carbamoyl-beta-alanine (3-ureidopropanoate) into beta-alanine, ammonia and carbon dioxide. Likewise, converts N-carbamoyl-beta-aminoisobutyrate (3-ureidoisobutyrate) into beta-aminoisobutyrate, ammonia and carbon dioxide.
Target Involvement
Beta-ureidopropionase deficiency (UPB1D)
Target Subcellular Location
Cytoplasm.
Target Protein Families
Carbon-nitrogen hydrolase superfamily, BUP family
Target Tissue Specificity
Detected in liver (at protein level).
Target Synonyms
UPB1; BUP1; Beta-ureidopropionase; EC 3.5.1.6; BUP-1; Beta-alanine synthase; N-carbamoyl-beta-alanine amidohydrolase
Target Background
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.
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