Overview

  • Product name

  • Description

    Rabbit polyclonal to PAH
  • Host species

    Rabbit
  • Tested applications

    Suitable for: IHC-P, WBmore details
  • Species reactivity

    Reacts with: Mouse, Human, Pig
  • Immunogen

    Recombinant fragment (His-tag) corresponding to Human PAH aa 1-226. (Expressed in E.coli).
    Sequence:

    MSTAVLENPGLGRKLSDFGQETSYIEDNCNQNGAISLIFSLKEEVGALAK VLRLFEENDVNLTHIESRPSRLKKDEYEFFTHLDKRSLPALTNIIKILRH DIGATVHELSRDKKKDTVPWFPRTIQELDRFANQILSYGAELDADHPGFK DPVYRARRKQFADIAYNYRHGQPIPRVEYMEEEKKTWGTVFKTLKSLYKT HACYEYNHIFPLLEKYCGFHEDNIPQ


    Database link: P00439

  • Positive control

    • IHC-P: Human liver and kidney tissue. WB: Human liver lysate; Pig kidney lysate; Mouse gallbladder lysate; HeLa cell lysate; Recombinant human PAH protein.

Properties

Applications

Our Abpromise guarantee covers the use of ab232999 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 Use a concentration of 5 - 20 µg/ml.
WB Use a concentration of 0.5 - 2 µg/ml. 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

  • Formalin-fixed, paraffin-embedded human liver tissue stained for PAH using ab232999 at 20 ug/ml in immunohistochemical analysis. DAB staining.

  • Formalin-fixed, paraffin-embedded human kidney tissue stained for PAH using ab232999 at 20 ug/ml in immunohistochemical analysis. DAB staining.

  • All lanes : Anti-PAH antibody (ab232999) at 2 µg/ml

    Lane 1 : Human liver tissue lysate
    Lane 2 : Pig kidney tissue lysate
    Lane 3 : Mouse gallbladder tissue lysate
    Lane 4 : HeLa (human epithelial cell line from cervix adenocarcinoma) cell lysate

    Secondary
    All lanes : HRP-Linked Guinea pig anti-rabbit at 1/2000 dilution

    Predicted band size: 52 kDa

  • Anti-PAH antibody (ab232999) at 2 µg/ml + Recombinant human PAH protein

    Secondary
    HRP-Linked Guinea pig anti-rabbit at 1/2000 dilution

    Predicted band size: 52 kDa

References

ab232999 has not yet been referenced specifically in any publications.

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