Overview

  • Product name

    Anti-PAH antibody [717CT21.1.1]
    See all PAH primary antibodies
  • Description

    Mouse monoclonal [717CT21.1.1] to PAH
  • Host species

    Mouse
  • Tested applications

    Suitable for: WBmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Human
    Predicted to work with: Cow
  • Immunogen

    Synthetic peptide within Human PAH aa 127-161 (internal sequence) conjugated to Keyhole Limpet Haemocyanin (KLH). The exact sequence is proprietary.
    Database link: P00439

  • Positive control

    • HepG2 cell line lysate; mouse liver and rat liver tissue lysates.

Properties

  • Form

    Liquid
  • Storage instructions

    Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
  • Storage buffer

    Preservative: 0.09% Sodium azide
    Constituent: 99% PBS
  • Concentration information loading...
  • Purity

    Protein G purified
  • Purification notes

    ab191234 was purified through a protein G column, followed by dialysis against PBS.
  • Clonality

    Monoclonal
  • Clone number

    717CT21.1.1
  • Isotype

    IgG1
  • Light chain type

    kappa
  • Research areas

Applications

Our Abpromise guarantee covers the use of ab191234 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. Predicted molecular weight: 52 kDa.

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 [717CT21.1.1] (ab191234) at 1/1000 dilution

    Lane 1 : HepG2 cell line lysate
    Lane 2 : mouse liver tissue lysate
    Lane 3 : rat liver tissue lysate

    Lysates/proteins at 35 µg per lane.

    Secondary
    All lanes : goat anti-mouse IgG H&L(HRP) at 1/3000 dilution

    Predicted band size: 52 kDa

References

ab191234 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab191234.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC PROCEDURES"
For licensing inquiries, please contact partnerships@abcam.com

Sign up