Overview

  • Product nameAnti-CPS1 antibodySee all CPS1 primary antibodies ...
  • Description
    Rabbit polyclonal to CPS1
  • 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, WBmore details
  • Species reactivity
    Reacts with: Mouse, Rat
  • Immunogen

    Full length native protein (purified) (Rat).

  • Positive control
    • Rat liver
  • General notesOMIM reference: 114010 Alternative name: carbamoylphosphate synthetase 1, CAD

Properties

Applications

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.

Application Abreviews Notes
IHC-Fr 1/100.
IHC-P 1/500.
ICC/IF Use at an assay dependent dilution.
IP Use at an assay dependent dilution.
WB Use at an assay dependent dilution.

Target

  • 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.
  • Information by UniProt
  • Database links
  • Alternative names
    • ammonia mitochondrial precursor antibody
    • Carbamoyl phosphate synthase [ammonia] antibody
    • Carbamoyl phosphate synthase [ammonia] mitochondrial antibody
    • Carbamoyl phosphate synthase antibody
    • Carbamoyl phosphate synthetase 1 antibody
    • Carbamoyl phosphate synthetase 1 mitochondrial antibody
    • Carbamoyl phosphate synthetase I antibody
    • Carbamoyl-phosphate synthase [ammonia] antibody
    • Carbamoyl-phosphate synthase antibody
    • carbamoyl-phosphate synthetase 1 mitochondrial antibody
    • Carbamoyl-phosphate synthetase I antibody
    • Carbamoylphosphate synthase antibody
    • Carbamoylphosphate synthetase 1 antibody
    • Carbamoylphosphate synthetase I antibody
    • cps 1 antibody
    • CPS1 antibody
    • CPSase 1 antibody
    • CPSase I antibody
    • CPSASE1 antibody
    • CPSM_HUMAN antibody
    • mitochondrial antibody
    • MS738 antibody
    see all

Anti-CPS1 antibody 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.

    See Abreview

  • 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.

    See Abreview

  • 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 (ab3682)

This product has been referenced in:
  • McGuire PJ  et al. Acute metabolic decompensation due to influenza in a mouse model of ornithine transcarbamylase deficiency. Dis Model Mech 7:205-13 (2014). WB ; Mouse . Read more (PubMed: 24271778) »
  • 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) »

See all 8 Publications for this product

Product Wall

Application Western blot
Loading amount 50 µg
Gel Running Conditions Reduced Denaturing (12%)
Sample Rat Tissue lysate - whole (Liver)
Specification Liver
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 21°C
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Submitted Nov 11 2013

Application Western blot
Loading amount 50 µg
Gel Running Conditions Reduced Denaturing (12%)
Sample Mouse Tissue lysate - whole (Liver)
Specification Liver
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 21°C
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Submitted Nov 07 2013

Application Immunocytochemistry/ Immunofluorescence
Sample Mouse Cell (Hepatocyte)
Specification Hepatocyte
Blocking step BSA as blocking agent for 30 minute(s) · Concentration: 2%
Fixative Formaldehyde
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Submitted Jun 12 2013

Application Western blot
Sample Rat Tissue lysate - whole (Liver)
Loading amount 50 µg
Specification Liver
Gel Running Conditions Reduced Denaturing (4-12%)
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 21°C
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Submitted Dec 25 2012

Application Western blot
Sample Mouse Tissue lysate - whole (Liver)
Loading amount 50 µg
Specification Liver
Gel Running Conditions Reduced Denaturing (4-12%)
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 21°C
Username

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Submitted Dec 25 2012

Vielen Dank für Ihren Anruf heute Morgen.
Ich habe mir die Beschreibung des IHC-P Bildes nochmals angeschaut und muss ihnen leider mitteilen, dass ich Ihnen keine genauen Angaben über dem Maleate Puffer machen kann, da dieses Bild und die Beschrei...

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Application Immunohistochemistry (Frozen sections)
Sample Rat Tissue sections (Liver)
Specification Liver
Fixative Acetone
Blocking step Serum as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 10% · Temperature: 22°C
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Submitted Jul 06 2012

Application Western blot
Sample Rat Tissue lysate - whole (liver)
Loading amount 40 µg
Specification liver
Treatment 17.5 mg/kg retrorsine for 24 h
Gel Running Conditions Reduced Non-Denaturing (Native) (12%)
Blocking step Milk as blocking agent for 1 hour(s) and 0 minute(s) · Concentration: 5% · Temperature: 25°C
Username

Dr. Junyi Xue

Verified customer

Submitted Jul 03 2012

Application Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample Mouse Tissue sections (liver E18.5)
Specification liver E18.5
Fixative Paraformaldehyde
Antigen retrieval step Heat mediated - Buffer/Enzyme Used: Citrate buffer
Permeabilization No
Blocking step (agent) for 30 minute(s) · Concentration: 100% · Temperature: 22°C
Username

Dr. Asha Seth

Verified customer

Submitted Jun 23 2009

Application Immunohistochemistry (Frozen sections)
Sample Mouse Tissue sections (Liver PO)
Specification Liver PO
Fixative Paraformaldehyde
Permeabilization No
Blocking step maleate blocking solution as blocking agent for 30 minute(s) · Concentration: 100% · Temperature: 22°C
Username

Dr. Asha Seth

Verified customer

Submitted Jun 23 2009

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