Principle of the Assay: This ELISA kit uses Sandwich-ELISA as the method. The micro ELISA plate provided in this kit has been pre-coated with an antibody specific to PAH. Standards or samples are added to the appropriate micro ELISA plate wells and combined with the specific antibody. Then a biotinylated detection antibody specific for PAH and Avidin-Horseradish Peroxidase (HRP) conjugate is added to each micro plate well successively and incubated. Free components are washed away. The substrate solution is added to each well. Only those wells that contain PAH, biotinylated detection antibody and Avidin-HRP conjugate will appear blue in color. The enzyme-substrate reaction is terminated by the addition of a sulphuric acid solution and the color turns yellow. The optical density (OD) is measured spectrophotometrically at a wavelength of 450 nm +/- 2 nm. The OD value is proportional to the concentration of PAH. You can calculate the concentration of PAH in the samples by comparing the OD of the samples to the standard curve.
NCBI and Uniprot Product Information
NCBI Description
PAH encodes the enzyme phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria. [provided by RefSeq, Jul 2008]
Uniprot Description
PAH: phenylalanine hydroxylase that is the rate-limiting step in phenylalanine catabolism. Belongs to the biopterin-dependent aromatic amino acid hydroxylase family. Deficiency of this enzyme activity results in the autosomal recessive disorder phenylketonuria.
Protein type: Amino Acid Metabolism - phenylalanine, tyrosine and tryptophan biosynthesis; Oxidoreductase; EC 1.14.16.1
Chromosomal Location of Human Ortholog: 12q22-q24.2
Cellular Component: cytosol
Molecular Function: amino acid binding; iron ion binding; phenylalanine 4-monooxygenase activity
Biological Process: L-phenylalanine catabolic process; catecholamine biosynthetic process; amino acid biosynthetic process; neurotransmitter biosynthetic process
Disease: Phenylketonuria
Research Articles on PAH
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Product Notes
The Rabbit PAH pah (Catalog #AAA2507746) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA2507746 ELISA Kit recognizes Rabbit PAH. It is sometimes possible for the material contained within the vial of "PAH, ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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