Product nameAnti-CPS1 antibody [EPR7494]
See all CPS1 primary antibodies
DescriptionRabbit monoclonal [EPR7494] to CPS1
Tested applicationsSuitable for: WB, IP, IHC-P, Flow Cyt, ICC/IFmore details
Species reactivityReacts with: Human
Synthetic peptide within Human CPS1 aa 1450-1550. The exact sequence is proprietary.
- HeLa cell lysate; Human fetal liver lysate; Human liver tissue
Mouse, Rat: We have preliminary internal testing data to indicate this antibody may not react with these species. Please contact us for more information.
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.
This product is a recombinant rabbit monoclonal antibody.
Storage instructionsShipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
Storage bufferpH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol, 0.05% BSA, 50% Tissue culture supernatant
PurityTissue culture supernatant
Our Abpromise guarantee covers the use of ab128942 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/1000 - 1/10000. Detects a band of approximately 150 kDa (predicted molecular weight: 165 kDa).|
|IP||1/10 - 1/100.|
|IHC-P||1/250 - 1/500. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.|
|Flow Cyt||Use at an assay dependent concentration.|
|ICC/IF||1/250 - 1/500.|
FunctionInvolved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
Tissue specificityPrimarily in the liver and small intestine.
Involvement in diseaseDefects in CPS1 are the cause of carbamoyl phosphate synthetase 1 deficiency (CPS1D) [MIM:237300]. CPS1D is an autosomal recessive disorder of the urea cycle causing hyperammonemia. Clinical features include protein intolerance, intermittent ataxia, seizures, lethargy, developmental delay and mental retardation.
Note=Genetic variations in CPS1 influence the availability of precursors for nitric oxide (NO) synthesis and play a role in clinical situations where endogenous NO production is critically important, such as neonatal pulmonary hypertension, increased pulmonary artery pressure following surgical repair of congenital heart defects or hepatovenocclusive disease following bone marrow transplantation. Infants with neonatal pulmonary hypertension homozygous for Thr-1406 have lower L-arginine concentrations than neonates homozygous for Asn-1406.
Sequence similaritiesContains 2 ATP-grasp domains.
Contains 1 glutamine amidotransferase type-1 domain.
DomainThe type-1 glutamine amidotransferase domain is defective.
- Information by UniProt
- Carbamoyl phosphate synthase [ammonia] antibody
- Carbamoyl phosphate synthase [ammonia] mitochondrial antibody
- Carbamoyl phosphate synthase antibody
All lanes : Anti-CPS1 antibody [EPR7494] (ab128942) at 1/1000 dilution
Lane 1 : HeLa cell lysate
Lane 2 : Human fetal liver tissue lysate
Lysates/proteins at 10 µg per lane.
All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution
Predicted band size: 165 kDa
Observed band size: 150 kDa why is the actual band size different from the predicted?
Flow Cytometry analysis of HeLa (human cervix adenocarcinoma) cells labeling CPS1 with unpurified ab128942 at 1/200 dilution (10ug/ml) (red). Cells were fixed with 4% paraformaldehyde and permeabilised with 90% methanol. A Goat anti-rabbit IgG (Alexa Fluor® 488)(1/2000 dilution) was used as the secondary antibody. Rabbit monoclonal IgG (Black) was used as the isotype control, cells without incubation with primary antibody and secondary antibody (Blue) were used as the unlabeled control.
ab128942, at 1/250 dilution, staining CPS1 in paraffin-embedded Human liver tissue by Immunohistochemistry.
ab128942 has not yet been referenced specifically in any publications.