Product nameAnti-CPS1 antibody
See all CPS1 primary antibodies
DescriptionRabbit polyclonal to CPS1
Tested applicationsSuitable for: WB, IHC-Fr, ICC/IF, IHC-Pmore details
Species reactivityReacts with: Mouse, Human
Predicted to work with: Rat
- WB: HeLa, A431 and HepG2 whole cell lysates.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Storage bufferPreservative: 0.02% Sodium Azide
Constituents: 1% BSA, PBS, pH 7.4
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab45956 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||Use a concentration of 1 µg/ml. Detects a band of approximately 150 kDa (predicted molecular weight: 140 kDa).|
|IHC-Fr||Use at an assay dependent concentration.|
|ICC/IF||Use a concentration of 1 µg/ml.|
|IHC-P||Use a concentration of 1 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.|
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 (ab45956) at 1 µg/ml
Lane 1 : HeLa (Human epithelial carcinoma cell line) Whole Cell Lysate
Lane 2 : A431 (Human epithelial carcinoma cell line) Whole Cell Lysate
Lane 3 : HepG2 (Human hepatocellular liver carcinoma cell line) Whole Cell Lysate
Lysates/proteins at 10 µg per lane.
All lanes : Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution
Performed under reducing conditions.
Predicted band size: 140 kDa
Observed band size: 150 kDa why is the actual band size different from the predicted?
ab45956 (1/500) staining CPS1 in HeLa cells (green). Cells were fixed in methanol and counterstained with DAPI in order to highlight the nucleus (red). Please refer to abreview for further experimental details.
ICC/IF image of ab45956 stained human HeLa cells. The cells were PFA fixed (10 min), permabilised in PBS-T (20 min) and incubated with the antibody (ab45956, 1µg/ml) for 1h at room temperature. 1%BSA / 10% normal goat serum / 0.3M glycine was used to quench autofluorescence and block non-specific protein-protein interactions. The secondary antibody (green) was Alexa Fluor® 488 goat anti-rabbit IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red). DAPI was used to stain the cell nuclei (blue).
IHC image of CPS1 staining in human liver carcinoma FFPE section, performed on a BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab45956, 1µg/ml, for 8 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.
ab45956 (1/200) staining CPS1 in mouse liver sections (red). Tissue sections were fixed in formaldehyde, blocked and then stained overnight at 4°C. Please refer to abreview for further experimental details.
This product has been referenced in:
- Burke ZD et al. Spatiotemporal regulation of liver development by the Wnt/ß-catenin pathway. Sci Rep 8:2735 (2018). WB . Read more (PubMed: 29426940) »
- Khoja S et al. Conditional disruption of hepatic carbamoyl phosphate synthetase 1 in mice results in hyperammonemia without orotic aciduria and can be corrected by liver-directed gene therapy. Mol Genet Metab N/A:N/A (2018). WB . Read more (PubMed: 29801986) »