Recombinant
RabMAb

Anti-PAH [EPR12381] antibody - C-terminal (ab191415)

Overview

  • Product name
    Anti-PAH [EPR12381] antibody - C-terminal
    See all PAH primary antibodies
  • Description
    Rabbit monoclonal [EPR12381] to PAH - C-terminal
  • Host species
    Rabbit
  • Tested applications
    Suitable for: Flow Cyt, WB, IHC-Pmore details
  • Species reactivity
    Reacts with: Mouse, Rat, Human
  • Immunogen

    Synthetic peptide within Human PAH aa 400 to the C-terminus (C terminal). The exact sequence is proprietary.
    Database link: P00439

  • Positive control
    • HeLa, 293T, HepG2, RAW 264.7, C6, NIH 3T3, PC12, Human fetal liver and U87-MG whole cell lysatse; Human liver and Mouse kidney tissues; U87-MG cells.
  • General notes

     

     

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

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab191415 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
Flow Cyt 1/300.

ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.

 

WB 1/1000 - 1/10000. Detects a band of approximately 52 kDa (predicted molecular weight: 52 kDa).
IHC-P 1/400 - 1/800. Perform heat mediated antigen retrieval with Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol.

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 [EPR12381] antibody - C-terminal (ab191415) at 1/1000 dilution

    Lane 1 : C6 cell lysate
    Lane 2 : RAW 264.7 cell lysate
    Lane 3 : PC12 cell lysate
    Lane 4 : NIH 3T3 cell lysate

    Lysates/proteins at 10 µg per lane.

    Secondary
    All lanes : Goat Anti-Rabbit IgG, (H+L),Peroxidase conjugated at 1/1000 dilution

    Predicted band size: 52 kDa

  • Flow cytometric analysis of U87-MG cells (paraformaldehyde-fixed, 2%) labeling PAH with ab191415 at 1/300 dilution (red), or a rabbit IgG (negative) (green), followed by Goat anti rabbit IgG (FITC) secondary at 1/150 dilution.

  • Immunohistochemical analysis of paraffin-embedded Mouse kidney tissue labeling PAH with ab191415 at 1/800 dilution (1.9 μg/ml) followed by pre-diluted HRP Polymer for Rabbit IgG secondary antibody and counter-stained with Hematoxylin.
    Inset: Negative control: using PBS instead of primary antibody.

  • All lanes : Anti-PAH [EPR12381] antibody - C-terminal (ab191415) at 1/10000 dilution

    Lane 1 : Human fetal liver lysate
    Lane 2 : HepG2 cell lysate
    Lane 3 : U87-MG cell lysate
    Lane 4 : 293 cell lysate

    Lysates/proteins at 20 µg per lane.

    Secondary
    All lanes : Goat Anti-Rabbit IgG, (H+L),Peroxidase conjugated at 1/1000 dilution

    Predicted band size: 52 kDa

  • Immunohistochemical analysis of paraffin-embedded Human liver tissue labeling PAH with ab191415 at 1/800 dilution (1.9 μg/ml)  followed by pre-diluted HRP Polymer for Rabbit IgG secondary antibody and counter-stained with Hematoxylin.
    Inset: Negative control: using PBS instead of primary antibody.

References

ab191415 has not yet been referenced specifically in any publications.

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

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