Key features and details
- Rabbit polyclonal to PAH
- Suitable for: IHC-P, WB
- Reacts with: Mouse, Human, Pig
Product nameAnti-PAH antibody
See all PAH primary antibodies
DescriptionRabbit polyclonal to PAH
Tested applicationsSuitable for: IHC-P, WBmore details
Species reactivityReacts with: Mouse, Human, Pig
Recombinant fragment (His-tag) corresponding to Human PAH aa 1-226. (Expressed in E.coli).
MSTAVLENPGLGRKLSDFGQETSYIEDNCNQNGAISLIFSLKEEVGALAK VLRLFEENDVNLTHIESRPSRLKKDEYEFFTHLDKRSLPALTNIIKILRH DIGATVHELSRDKKKDTVPWFPRTIQELDRFANQILSYGAELDADHPGFK DPVYRARRKQFADIAYNYRHGQPIPRVEYMEEEKKTWGTVFKTLKSLYKT HACYEYNHIFPLLEKYCGFHEDNIPQ
Database link: P00439
- IHC-P: Human liver and kidney tissue. WB: Human liver lysate; Pig kidney lysate; Mouse gallbladder lysate; HeLa cell lysate; Recombinant human PAH protein.
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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.
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Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferpH: 7.40
Preservative: 0.02% Sodium azide
Constituents: PBS, 50% Glycerol
Concentration information loading...
PurityImmunogen affinity purified
Purification notesAntigen-specific affinity chromatography followed by Protein A affinity chromatography.
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.
|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.|
PathwayAmino-acid degradation; L-phenylalanine degradation; acetoacetate and fumarate from L-phenylalanine: step 1/6.
Involvement in diseaseDefects 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 similaritiesBelongs to the biopterin-dependent aromatic amino acid hydroxylase family.
Contains 1 ACT domain.
- Information by UniProt
- PAH antibody
- PH antibody
- PH4H_HUMAN antibody
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
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
HRP-Linked Guinea pig anti-rabbit at 1/2000 dilution
Predicted band size: 52 kDa
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
ab232999 has not yet been referenced specifically in any publications.