Overview

  • Product nameAnti-CPS1 antibody
    See all CPS1 primary antibodies
  • Description
    Rabbit polyclonal to CPS1
  • Tested applicationsSuitable for: WBmore details
  • Species reactivity
    Reacts with: Human
  • Immunogen

    Synthetic peptide conjugated to KLH, corresponding to a region within N-terminal amino acids 261-291 of Human CPS1 (NP_001116105.1, NP_001866.2, NP_001116106.1).

  • Positive control
    • Hela cell line lysate

Properties

Applications

Our Abpromise guarantee covers the use of ab123859 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
WB 1/100 - 1/500. Predicted molecular weight: 165 kDa.

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
    • 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
    • CPSASE1 antibody
    • CPSM_HUMAN antibody
    • mitochondrial antibody
    • MS738 antibody
    see all

Anti-CPS1 antibody images

  • Anti-CPS1 antibody (ab123859) at 1/100 dilution + Hela cell line lysate at 35 µg

    Predicted band size : 165 kDa

References for Anti-CPS1 antibody (ab123859)

ab123859 has not yet been referenced specifically in any publications.

Product Wall

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