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Anti-CPS1 antibody [2E4AE11 ] (ab110303)

MSCatalog No. MS738

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Overview

Product name

Anti-CPS1 antibody [2E4AE11 ]
See all CPS1 products (8) ...

Description

Mouse monoclonal [2E4AE11 ] to CPS1

Tested applications

IHC-P, Flow Cyt, ICC, IPmore details

Cross reactivity

Reacts with

Human

Positive control

HDFn and HeLa cells; Human liver mitochondria lysate.

Properties

Form

Liquid

Storage instructions

Store at +4°C. Do not freeze.

Storage buffer

Preservative: 0.02% Sodium azide
Constituent: HBS

Concentration

Concentration information loading...

Purity

>95% by SDS-PAGE

Purification notes

ab110303 was produced in vitro using hybridomas grown in serum-free medium, and then purified by affinity purification

Clonality

Monoclonal

Clone number

2E4AE11

Isotype

IgG1

Light chain type

kappa

  • Immunocytochemistry - CPS1 antibody [2E4AE11 ] (ab110303)Immunocytochemistry - CPS1 antibody [2E4AE11 ] (ab110303) image (enlarge)

  • Immunoprecipitation - CPS1 antibody [2E4AE11 ] (ab110303)Immunoprecipitation - CPS1 antibody [2E4AE11 ] (ab110303) image (enlarge)

  • Flow Cytometry - CPS1 antibody [2E4AE11 ] (ab110303)Flow Cytometry - CPS1 antibody [2E4AE11 ] (ab110303) image (enlarge)

Applications

Show applications key

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.

Target

Function

Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.

Tissue specificity

Primarily in the liver and small intestine.

Involvement in disease

Defects 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 similarities

Contains 2 ATP-grasp domains.
Contains 1 glutamine amidotransferase type-1 domain.

Domain

The type-1 glutamine amidotransferase domain is defective.

Cellular localization

Mitochondrion.

Target information above from: UniProt accessionP31327 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010).

Information by UniProt

Alternative names

  • mitochondrial 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 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
  • CPSM_HUMAN antibody
  • MS738 antibody
see all

Anti-CPS1 antibody [2E4AE11 ] images:

  Immunocytochemistry - CPS1 antibody [2E4AE11 ] (ab110303)

Immunocytochemistry - CPS1 antibody [2E4AE11 ] (ab110303)

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.

  Immunoprecipitation - CPS1 antibody [2E4AE11 ] (ab110303)

Immunoprecipitation - CPS1 antibody [2E4AE11 ] (ab110303)

Detection of ab110303 by immunoprecipitation staining of 164kDa CPS1, a liver mitochondrial marker in human liver mitochondrial lysate.

  Flow Cytometry - CPS1 antibody [2E4AE11 ] (ab110303)

Flow Cytometry - CPS1 antibody [2E4AE11 ] (ab110303)

Flow cytometric analysis using ab110303 at 1µg/ml staining CPS1 in HeLa cells (blue). Isotype control antibody (red).

  Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-CPS1 antibody [2E4AE11 ] (ab110303)

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-CPS1 antibody [2E4AE11 ] (ab110303)

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.

References for Anti-CPS1 antibody [2E4AE11 ] (ab110303)

ab110303 has not yet been referenced specifically in any publications.

Publishing research using ab110303? Please let us know so that we can cite the reference in this datasheet

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"