Anti-CPS1 antibody - Liver Mitochondrial Marker (ab3682)
- Product nameAnti-CPS1 antibody - Liver Mitochondrial MarkerSee all CPS1 primary antibodies ...
- DescriptionRabbit polyclonal to CPS1 - Liver Mitochondrial Marker
- SpecificityWhen the antibody is used at 1/2000 some lower molecular weight bands appear in COS-7 cells, the extent of which is significantly reduced when the antibody is used at 1/10,000.
- Tested applicationsIHC-Fr, IHC-P, ICC/IF, IP, WB more details
- Species reactivityReacts with: Mouse, Rat
Full length native protein (purified) (Rat).
- Positive controlRat liver
- General notesOMIM reference: 114010 Alternative name: carbamoylphosphate synthetase 1, CAD
- Storage instructionsStore at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C (add glycerol to a final volume of 50% for extra stability). Avoid repeated freeze / thaw cycles.
- Storage bufferserum with 0.02% sodium azide
- PurityWhole antiserum
- Clonality Polyclonal
Our Abpromise guarantee covers the use of ab3682 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|ICC/IF||ICC/IF: Use at an assay dependent dilution.|
|IP||IP: Use at an assay dependent dilution.|
|WB||WB: Use at an assay dependent dilution.|
- 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.
- Cellular localizationMitochondrion.
- ammonia mitochondrial precursor antibodyCarbamoyl phosphate synthase [ammonia] antibodyCarbamoyl phosphate synthase [ammonia] mitochondrial antibody
- Carbamoyl phosphate synthase antibodyCarbamoyl phosphate synthetase 1 antibodyCarbamoyl phosphate synthetase 1 mitochondrial antibodyCarbamoyl phosphate synthetase I antibodyCarbamoyl-phosphate synthase [ammonia] antibodyCarbamoyl-phosphate synthase antibodycarbamoyl-phosphate synthetase 1 mitochondrial antibodyCarbamoyl-phosphate synthetase I antibodyCarbamoylphosphate synthase antibodyCarbamoylphosphate synthetase 1 antibodyCarbamoylphosphate synthetase I antibodycps 1 antibodyCPS1 antibodyCPSase 1 antibodyCPSase I antibodyCPSASE1 antibodyCPSM_HUMAN antibodymitochondrial antibodyMS738 antibody
Anti-CPS1 antibody - Liver Mitochondrial Marker images
Ab3682 can IP CPS1 transiently expressed in COS-7 cells.
Lane 1: 5 ul of 2ml lysate cos-7 cells transfected with full length CPS 1. Before IP
Lane 2: cos-7 cells transfected with full length CPS 1. IP with 5 ul of pre-immune serum
Lane 3: cos-7 cells transfected with full length CPS 1. IP with 2 ul of polyclonal antibody anti CPS 1 (ab3682)
Lane 4: cos-7 cells transfected with full length CPS 1. IP with 5 ul of polyclonal antibody anti CPS 1 (ab3682)
Lane 5: 5 ul of 2ml lysate cos-7 cells transfected with empty vector (pCMV5-tr). Before IP
Lane 6: cos-7 cells transfected with empty vector (pCMV5-tr). IP with 5 ul of polyclonal antibody anti CPS 1 (ab3682)
Lane 7: 5 ul of 2ml lysate untransfected cos-7 cells. Before IP
Lane 8: untransfected cos-7 cells. IP with 5 ul of polyclonal antibody anti CPS 1 (ab3682)
CPS1 - Liver Mitochondrial Marker was immunoprecipitated using 0.5mg Hela whole cell extract, 5µg of Rabbit polyclonal to CPS1 - Liver Mitochondrial Marker (ab3682) and 50µl of protein G magnetic beads (+). No antibody was added to the control (-).
The antibody was incubated under agitation with Protein G beads for 10min, Hela whole cell extract lysate diluted in RIPA buffer was added to each sample and incubated for a further 10min under agitation.
Proteins were eluted by addition of 40µl SDS loading buffer and incubated for 10min at 70oC; 10µl of each sample was separated on a SDS PAGE gel, transferred to a nitrocellulose membrane, blocked with 5% BSA and probed with ab3682.
Secondary: Antibody to Rabbit IgG light chain (HRP) (ab99697).
Band: 165kDa: CPS1.
ab3682 staining CPS1 in mouse hepatic tissue section by Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections). Tissue underwent paraformaldehyde fixation before heat mediated antigen retrieval in Citrate buffer and then blocked in maleate blocking solution for 30 minutes at 22°C. The primary antibody was diluted 1/500 and incubated with sample in maleate buffer blocking solution for 16 hours at 22°C. A Biotin conjugated donkey polyclonal to rabbit IgG was used at dilution at 1/250 as secondary antibody.
ab3682 staining CPS1 in mouse liver tissue section by Immunohistochemistry (Frozen sections). Tissue samples were fixed with paraformaldehyde and blocking with 100% maleate blocking solution at 220C for 30 minutes was performed. The sample was incubated with primary antibody (1/100) at 220C for 16 hours. An Alexa Fluor®488-conjugated donkey polyclonal to rabbit IgG was used undiluted as a secondary antibody. The image demonstrates CPS1 in green, nuclei stained blue with DAPI.
CPS-1 localisation is consistent with the reticulated pattern of liver mitonchondria.
Rabbit polyclonal to CPS-1 (ab3682), at 1/5000 on COS-7 cells transiently transfected with pCMV5-CPS 1 plasmid (Corvi, M.M and Berthiaume, L.G. unpublished data).
Secondary: donkey anti-rabbit (Texas Red).
Western blot using Rabbit polyclonal to CPS 1 (ab3682).
All lanes loaded with 100 ng of purified CPS 1
Lane 1: ab3682 1/500
Lane 2: ab3682 1/1000
Lane 3: ab3682 1/2000
References for Anti-CPS1 antibody - Liver Mitochondrial Marker (ab3682)
This product has been referenced in:
- Wilson CH et al. Increased expression of peroxiredoxin 1 and identification of a novel lipid-metabolizing enzyme in the early phase of liver ischemia reperfusion injury. Proteomics 11:4385-96 (2011). WB ; Rat . Read more (PubMed: 21905220) »
- Walters MW & Wallace KB Urea cycle gene expression is suppressed by PFOA treatment in rats. Toxicol Lett 197:46-50 (2010). Read more (PubMed: 20452409) »