Overview

  • Product name
  • Description
    Goat polyclonal to PAH
  • Tested applications
    Suitable for: WBmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Human
    Predicted to work with: Chicken, Cow, Dog
  • Immunogen

    Synthetic peptide:

    ESRPSRLKKDE

    by a Cysteine residue linker, corresponding to internal sequence amino acids 66-76 of Human PAH (NP_000268.1).

  • Positive control
    • Human Liver lysates

Properties

Applications

Our Abpromise guarantee covers the use of ab106805 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 Use a concentration of 0.3 - 1 µg/ml. Detects a band of approximately 48 kDa (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 (ab106805) at 0.10000000149 µg/ml

    Lane 1 : Mouse Liver lysate in RIPA buffer
    Lane 2 : Rat Liver lysate in RIPA buffer

    Lysates/proteins at 35 µg per lane.

    Developed using the ECL technique

    Predicted band size : 52 kDa
  • Anti-PAH antibody (ab106805) at 0.3 µg/ml + Human Liver lysate (in RIPA buffer) at 35 µg
    Developed using the ECL technique

    Predicted band size : 52 kDa
    Observed band size : 48 kDa (why is the actual band size different from the predicted?)

References

ab106805 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab106805.
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