Recombinant
RabMAb

Anti-PAH antibody [EPR12380] (ab178430)

Overview

  • Product name
    Anti-PAH antibody [EPR12380]
    See all PAH primary antibodies
  • Description
    Rabbit monoclonal [EPR12380] to PAH
  • Tested applications
    Suitable for: WB, IHC-P, ICC/IF, Flow Cytmore details
    Unsuitable for: IP
  • Species reactivity
    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide (the amino acid sequence is considered to be commercially sensitive) within Human PAH aa 50-150 (Cysteine residue). The exact sequence is proprietary.
    Database link: P00439

  • Positive control
    • Human kidney and liver tissues; Human fetal liver tissue lysate and HepG2 cell line lysate.
  • General notes

    If you have any questions regarding this update, please contact our Scientific Support team.

     

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents

    We are constantly working hard to ensure we provide our customers with best in class antibodies. As a result of this work we are pleased to now offer this antibody in purified format. We are in the process of updating our datasheets. The purified format is designated 'PUR' on our product labels. If you have any questions regarding this update, please contact our Scientific Support team.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab178430 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 1/1000 - 1/10000. Predicted molecular weight: 51 kDa.
IHC-P 1/50 - 1/100. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
ICC/IF 1/50 - 1/100.
Flow Cyt 1/10 - 1/100. ab172730-Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.
  • Application notes
    Is unsuitable for IP.
  • Target

    • Pathway
      Amino-acid degradation; L-phenylalanine degradation; acetoacetate and fumarate from L-phenylalanine: step 1/6.
    • Involvement in disease
      Defects in PAH are the cause of phenylketonuria (PKU) [MIM:261600]. PKU is an autosomal recessive inborn error of phenylalanine metabolism, due to severe phenylalanine hydroxylase deficiency. It is characterized by blood concentrations of phenylalanine persistently above 1200 mumol (normal concentration 100 mumol) which usually causes mental retardation (unless low phenylalanine diet is introduced early in life). They tend to have light pigmentation, rashes similar to eczema, epilepsy, extreme hyperactivity, psychotic states and an unpleasant 'mousy' odor.
      Defects in PAH are the cause of non-phenylketonuria hyperphenylalaninemia (Non-PKU HPA) [MIM:261600]. Non-PKU HPA is a mild form of phenylalanine hydroxylase deficiency characterized by phenylalanine levels persistently below 600 mumol, which allows normal intellectual and behavioral development without treatment. Non-PKU HPA is usually caused by the combined effect of a mild hyperphenylalaninemia mutation and a severe one.
      Defects in PAH are the cause of hyperphenylalaninemia (HPA) [MIM:261600]. HPA is the mildest form of phenylalanine hydroxylase deficiency.
    • Sequence similarities
      Belongs to the biopterin-dependent aromatic amino acid hydroxylase family.
      Contains 1 ACT domain.
    • Information by UniProt
    • Database links
    • Alternative names
      • PAH antibody
      • PH antibody
      • PH4H_HUMAN antibody
      • Phe 4 monooxygenase antibody
      • Phe-4-monooxygenase antibody
      • Phenylalanine 4 hydroxylase antibody
      • Phenylalanine hydroxylase antibody
      • Phenylalanine-4-hydroxylase antibody
      • PKU antibody
      • PKU1 antibody
      see all

    Images

    • All lanes : Anti-PAH antibody [EPR12380] (ab178430) at 1/1000 dilution

      Lane 1 : Human fetal liver lysate
      Lane 2 : HepG2 cell line lysate

      Lysates/proteins at 10 µg per lane.

      Secondary
      Goat anti-rabbit HRP conjugated antibody
      Developed using the ECL technique

      Predicted band size : 51 kDa
    • Immunofluorescent analysis of HepG2 cells labeling PAH with ab178430 at 1/50 dilution (green). DAPI nuclear staining (blue).

    • Flow cytometric analysis of permeabilized HepG2 cells labeling PAH with ab178430 at 1/10 dilution (red) or a rabbit IgG (negative) (green).

    • Immunohistochemical analysis of paraffin-embedded Human liver tissue labeling PAH with ab178430 at 1/50 dilution.

    • Immunohistochemical analysis of paraffin-embedded Human kidney tissue labeling PAH with ab178430 at 1/50 dilution.

    References

    ab178430 has not yet been referenced specifically in any publications.

    Customer reviews and Q&As

    There are currently no Customer reviews or Questions for ab178430.
    Please use the links above to contact us or submit feedback about this product.

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

    Sign up