Key features and details
- Mouse monoclonal [2E4AE11] to CPS1
- Suitable for: IHC-P, Flow Cyt, ICC, IP
- Reacts with: Human
- Isotype: IgG1
Product nameAnti-CPS1 antibody [2E4AE11]
See all CPS1 primary antibodies
DescriptionMouse monoclonal [2E4AE11] to CPS1
Tested applicationsSuitable for: IHC-P, Flow Cyt, ICC, IPmore details
Species reactivityReacts with: Human
Tissue, cells or virus. This information is considered to be commercially sensitive.
- HDFn and HeLa cells; Human liver mitochondria lysate.
This antibody clone is manufactured by Abcam.
Immunogen information: Human liver mitochodnria was used to generate this antibody. The exact immunogen sequence is unknown.
Product was previously marketed under the MitoSciences sub-brand.
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Abcam is leading the way with our range of recombinant antibodies, knockout-validated antibodies and knockout cell lines, all of which support improved reproducibility.
We are also planning to innovate the way in which we present recommended applications and species on our product datasheets, so that only applications & species that have been tested in our own labs, our suppliers or by selected trusted collaborators are covered by our Abpromise™ guarantee.
In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.
We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.
Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.
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Storage instructionsShipped at 4°C. Store at +4°C. Do Not Freeze.
Storage bufferPreservative: 0.02% Sodium azide
Constituent: HEPES buffered saline
Concentration information loading...
Purification notesab110303 was produced in vitro using hybridomas grown in serum-free medium. Purity: >95% by SDS-PAGE.
Light chain typekappa
Our Abpromise guarantee covers the use of ab110303 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||Use a concentration of 10 µg/ml. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.|
|Flow Cyt||Use a concentration of 1 µg/ml.
ab170190 - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.
|ICC||Use a concentration of 10 µg/ml.|
|IP||Use at an assay dependent concentration.|
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
Flow cytometric analysis using ab110303 at 1µg/ml staining CPS1 in HeLa cells (blue). Isotype control antibody (red).
Immunocytochemistry analysis using ab110303 at 10µg/ml staining CPS1 in HDFn cells (paraformaldehyde fixed and Triton X-100 permeabilized). The secondary antibody was (red) Alexa Fluor® 594 goat anti-mouse IgG (H+L) used at a 1/1000 dilution for 1h. 10% Goat serum was used as the blocking agent for all blocking steps. DAPI was used to stain the cell nuclei (blue). Target protein locates mainly in mitochondria.
Detection of ab110303 by immunoprecipitation staining of 164kDa CPS1, a liver mitochondrial marker in human liver mitochondrial lysate.
IHC image of ab110303 staining in human duodenum formalin fixed paraffin embedded tissue section, performed on a Leica 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 ab110303, 10µg/ml, for 15 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.
For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.
ab110303 has been referenced in 1 publication.
- Li J et al. Detection of circulating tumor cells in hepatocellular carcinoma using antibodies against asialoglycoprotein receptor, carbamoyl phosphate synthetase 1 and pan-cytokeratin. PLoS One 9:e96185 (2014). Flow Cyt ; Human . PubMed: 24763545