Product nameAnti-PAH antibody [EPR12380] (Alexa Fluor® 647)
See all PAH primary antibodies
DescriptionRabbit monoclonal [EPR12380] to PAH (Alexa Fluor® 647)
ConjugationAlexa Fluor® 647. Ex: 652nm, Em: 668nm
Tested applicationsSuitable for: Flow Cytmore details
Species reactivityReacts with: Human
Predicted to work with: Mouse, Rat
Synthetic peptide within Human PAH aa 50-150 (Cysteine residue). The exact sequence is proprietary.
Database link: P00439
- Flow Cyt: HeLa cells.
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.
Alexa Fluor® is a registered trademark of Molecular Probes, Inc, a Thermo Fisher Scientific Company. The Alexa Fluor® dye included in this product is provided under an intellectual property license from Life Technologies Corporation. As this product contains the Alexa Fluor® dye, the purchase of this product conveys to the buyer the non-transferable right to use the purchased product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). As this product contains the Alexa Fluor® dye the sale of this product is expressly conditioned on the buyer not using the product or its components, or any materials made using the product or its components, in any activity to generate revenue, which may include, but is not limited to use of the product or its components: in manufacturing; (ii) to provide a service, information, or data in return for payment (iii) for therapeutic, diagnostic or prophylactic purposes; or (iv) for resale, regardless of whether they are sold for use in research. For information on purchasing a license to this product for purposes other than research, contact Life Technologies Corporation, 5781 Van Allen Way, Carlsbad, CA 92008 USA or firstname.lastname@example.org.
This product is a recombinant rabbit monoclonal antibody.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C. Stable for 12 months at -20°C. Store In the Dark.
Storage bufferpH: 7.4
Preservative: 0.02% Sodium azide
Constituents: PBS, 30% Glycerol, 1% BSA
Concentration information loading...
PurityProtein A purified
Our Abpromise guarantee covers the use of ab207388 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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
Overlay histogram showing HeLa cells stained with ab207388 (red line). The cells were fixed with 4% formaldehyde (10 min) and then permeabilized with 90% methanol for 30 min at -20°C. The cells were then incubated in 1x PBS / 10% normal goat serum to block non-specific protein-protein interactions followed by the antibody (ab207388, 1/50 dilution) for 30 min at 22°C.
Isotype control antibody (black line) was rabbit IgG (monoclonal) Alexa Fluor® 647 (ab199093) used at the same concentration and conditions as the primary antibody. Unlabelled sample (blue line) was also used as a control.
Acquisition of >5,000 events were collected using a 40mW Red laser (640nm) and 670/14 bandpass filter.
ab207388 has not yet been referenced specifically in any publications.