Overview

  • Product name

  • Description

    Rabbit polyclonal to PAH
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, WB, ICC/IFmore details
  • Species reactivity

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

    Recombinant fragment corresponding to Human PAH aa 2-452.
    Sequence:

    STAVLENPGLGRKLSDFGQETSYIEDNCNQNGAISLIFSLKEEVGALAKV LRLFEENDVNLTHIESRPSRLKKDEYEFFTHLDKRSLPALTNIIKILRHD IGATVHELSRDKKKDTVPWFPRTIQELDRFANQILSYGAELDADHPGFKD PVYRARRKQFADIAYNYRHGQPIPRVEYMEEEKKTWGTVFKTLKSLYKTH ACYEYNHIFPLLEKYCGFHEDNIPQLEDVSQFLQTCTGFRLRPVAGLLSS RDFLGGLAFRVFHCTQYIRHGSKPMYTPEPDICHELLGHVPLFSDRSFAQ FSQEIGLASLGAPDEYIEKLATIYWFTVEFGLCKQGDSIKAYGAGLLSSF GELQYCLSEKPKLLPLELEKTAIQNYTVTEFQPLYYVAESFNDAKEKVRN FAATIPRPFSVRYDPYTQRIEVLDNTQQLKILADSINSEIGILCSALQKI K


    Database link: P00439

  • Positive control

    • WB: Mouse kidney, liver, heart and brain tissue lysate. RAW 264.7 whole cell lysate. IHC-P: Human kidney tissue. ICC/IF: HepG2 cells.

Properties

Applications

Our Abpromise guarantee covers the use of ab237487 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-P 1/50 - 1/200.
WB 1/2000 - 1/5000.
ICC/IF 1/20 - 1/200.

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 (ab237487) at 1/500 dilution

    Lane 1 : RAW 264.7 (Mouse macrophage cell line transformed with Abelson murine leukemia virus) whole cell lysate
    Lane 2 : Mouse heart tissue lysate
    Lane 3 : Mouse brain tissue lysate

    Secondary
    All lanes : Goat polyclonal to rabbit IgG at 1/50000 dilution
  • Paraffin-embedded human kidney tissue stained for PAH using ab237487 at 1/100 dilution in immunohistochemical analysis.

  • HepG2 (Human liver hepatocellular carcinoma cell line) cells stained for PAH (Green) using ab237487 at 1/100 dilution in iCC/IF, followed by an Alexa-Fluor®488-conjugated Goat Anti-Rabbit IgG (H+L) secondary antibody.

  • HepG2 (Human liver hepatocellular carcinoma cell line) cells stained for PAH (Green) using ab237487 at 1/100 dilution in iCC/IF, followed by an Alexa-Fluor®488-conjugated Goat Anti-Rabbit IgG (H+L) secondary antibody.

  • All lanes : Anti-PAH antibody (ab237487) at 1/500 dilution

    Lane 1 : Mouse kidney tissue lysate
    Lane 2 : Mouse liver tissue lysate

    Secondary
    All lanes : Goat polyclonal to Rabbit IgG at 1/10000 dilution

References

ab237487 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab237487.
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