RecombinantRabMAb

Recombinant Anti-PAH antibody [EPR12380] (ab178430)

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Western blot - Anti-PAH antibody [EPR12380] (ab178430)
  • Immunocytochemistry/ Immunofluorescence - Anti-PAH antibody [EPR12380] (ab178430)
  • Flow Cytometry - Anti-PAH antibody [EPR12380] (ab178430)
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-PAH antibody [EPR12380] (ab178430)
  • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-PAH antibody [EPR12380] (ab178430)
  • Anti-PAH antibody [EPR12380] (ab178430)

Key features and details

  • Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
  • Rabbit monoclonal [EPR12380] to PAH
  • Suitable for: WB, IHC-P, ICC/IF, Flow Cyt
  • Reacts with: Mouse, Rat, Human

Overview

  • Product name

    Anti-PAH antibody [EPR12380]
    See all PAH primary antibodies

  • Description

    Rabbit monoclonal [EPR12380] to PAH

  • Host species

    Rabbit

  • Tested applications

    Suitable for: WB, IHC-P, ICC/IF, Flow Cytmore details
    Unsuitable for: IP

  • Species reactivity

    Reacts with: Mouse, Rat, Human

  • Immunogen

    Synthetic peptide within Human PAH aa 50-150 (Cysteine residue). The exact sequence is proprietary.
    Database link: P00439

  • Positive control

    • Human kidney and liver tissues; Human fetal liver tissue lysate and HepG2 cell line lysate.

  • General notes

    This product is a recombinant monoclonal antibody, which offers several advantages including:

    • - High batch-to-batch consistency and reproducibility
    • - Improved sensitivity and specificity
    • - Long-term security of supply
    • - Animal-free production
    For more information see here.

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

    We are constantly working hard to ensure we provide our customers with best in class antibodies. As a result of this work we are pleased to now offer this antibody in purified format. We are in the process of updating our datasheets. The purified format is designated 'PUR' on our product labels. If you have any questions regarding this update, please contact our Scientific Support team.

    Reproducibility is key to advancing scientific discovery and accelerating scientists’ next breakthrough.

    Abcam is leading the way with our range of recombinant antibodies, knockout-validated antibodies and knockout cell lines, all of which support improved reproducibility.

    We are also planning to innovate the way in which we present recommended applications and species on our product datasheets, so that only applications & species that have been tested in our own labs, our suppliers or by selected trusted collaborators are covered by our Abpromise™ guarantee.

    In preparation for this, we have started to update the applications & species that this product is Abpromise guaranteed for.

    We are also updating the applications & species that this product has been “predicted to work with,” however this information is not covered by our Abpromise guarantee.

    Applications & species from publications and Abreviews that have not been tested in our own labs or in those of our suppliers are not covered by the Abpromise guarantee.

    Please check that this product meets your needs before purchasing. If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, as well as customer reviews and Q&As.

Properties

Applications

Our Abpromise guarantee covers the use of ab178430 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 - 1/10000. Predicted molecular weight: 51 kDa.
IHC-P 1/50 - 1/100. Perform heat mediated antigen retrieval before commencing with IHC staining protocol.
ICC/IF 1/50 - 1/100.
Flow Cyt 1/10 - 1/100.

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

 
  • Application notes
    Is unsuitable for IP.

    • 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

      see all

    • 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

    • Western blot - Anti-PAH antibody [EPR12380] (ab178430)
      Western blot - Anti-PAH antibody [EPR12380] (ab178430)
      All lanes : Anti-PAH antibody [EPR12380] (ab178430) at 1/1000 dilution

      Lane 1 : Human fetal liver lysate
      Lane 2 : HepG2 cell line lysate

      Lysates/proteins at 10 µg per lane.

      Secondary
      All lanes : Goat anti-rabbit HRP conjugated antibody

      Developed using the ECL technique.

      Predicted band size: 51 kDa

    • Immunocytochemistry/ Immunofluorescence - Anti-PAH antibody [EPR12380] (ab178430)
      Immunocytochemistry/ Immunofluorescence - Anti-PAH antibody [EPR12380] (ab178430)

      Immunofluorescent analysis of HepG2 cells labeling PAH with ab178430 at 1/50 dilution (green). DAPI nuclear staining (blue).

    • Flow Cytometry - Anti-PAH antibody [EPR12380] (ab178430)
      Flow Cytometry - Anti-PAH antibody [EPR12380] (ab178430)

      Flow cytometric analysis of permeabilized HepG2 cells labeling PAH with ab178430 at 1/10 dilution (red) or a rabbit IgG (negative) (green).

    • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-PAH antibody [EPR12380] (ab178430)
      Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-PAH antibody [EPR12380] (ab178430)

      Immunohistochemical analysis of paraffin-embedded Human liver tissue labeling PAH with ab178430 at 1/50 dilution.

      Perform heat mediated antigen retrieval before commencing with IHC staining protocol.

    • Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-PAH antibody [EPR12380] (ab178430)
      Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-PAH antibody [EPR12380] (ab178430)

      Immunohistochemical analysis of paraffin-embedded Human kidney tissue labeling PAH with ab178430 at 1/50 dilution.

      Perform heat mediated antigen retrieval before commencing with IHC staining protocol.

    • Anti-PAH antibody [EPR12380] (ab178430)
      Anti-PAH antibody [EPR12380] (ab178430)

    References (1)

    Publishing research using ab178430? Please let us know so that we can cite the reference in this datasheet.

    ab178430 has been referenced in 1 publication.

    • Zhang X  et al. In vitro residual activities in 20 variants of phenylalanine hydroxylase and genotype-phenotype correlation in phenylketonuria patients. Gene 707:239-245 (2019). PubMed: 31102715

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