Product Name:
PAH-pS16
Product Number:
ab-pn713
Target Full Name: Phenylalanine-4-hydroxylase
Target Alias: Phe-4-monooxygenase
Product Type Specific: PAH phosphosite-specific antibody
Antibody Code: PN713
Antibody Target Type: Phosphosite-specific
Antibody Phosphosite: S16
Protein UniProt: P00439
Protein SigNET: PAH
Antibody Type: Polyclonal
Antibody Host Species: Rabbit
Antibody Immunogen Source: Synthetic phosphopeptide patterned after human PAH
Antibody Immunogen Sequence: RKL(pS)DFG(βA)C
Antibody Immunogen Description: Corresponds to amino acid residues R13 to G19. S16 phosphorylation stimulates enzyme activity and inhibits interaction with PAH. This is the major in vivo phosphorylation sites in phenylalanine-4-hydroxylase (≥41 reports from high throughput mass spectrometry studies recorded in PhosphoSitePlus). This human phosphosite is not highly conserved in vertebrates, but is found in the rat. PAH is known to be phosphorylated at this site in vitro by PKACa (PRKACA).
Production Method: The immunizing peptide was produced by solid phase synthesis on a multipep peptide synthesizer and purified by reverse-phase hplc chromatography. Purity was assessed by analytical hplc and the amino acid sequence confirmed by mass spectrometry analysis. This peptide was coupled to KLH prior to immunization into rabbits. New Zealand White rabbits were subcutaneously injected with KLH-coupled immunizing peptide every 4 weeks for 4 months. The sera from these animals was applied onto an agarose column to which the immunogen peptide was thio-linked. Antibody was eluted from the column with 0.1 M glycine, pH 2.5. Subsequently, the antibody solution was neutralized to pH 7.0 with saturated Tris.
Antibody Modification: Unconjugated. Contact KInexus if you are interest in having the antibody biotinylated or coupled with fluorescent dyes.
Antibody Concentration: 0.8 mg/ml
Storage Buffer: Phosphate buffered saline pH 7.4, 0.05% Thimerasol
Storage Conditions: For long term storage, keep frozen at -40°C or lower. Stock solution can be kept at +4°C for more than 3 months. Avoid repeated freeze-thaw cycles.
Product Use: Western blotting | Antibody microarray
Antibody Dilution Recommended: 2 µg/ml for immunoblotting
Antibody Species Reactivity: This antibody detects the target phosphoprotein in the following species due to conservation of amino acid sequence: Human | Chimpanzee | Rat | Mouse.
Scientific Background: PAH (Phenylalanine hydroxylase) is a critical liver enzyme that belongs to the biopterin-dependent aromatic amino acid hydroxylase family. It catalyzes the rate-limiting step in phenylalanine catabolism, converting the essential amino acid L-phenylalanine into L-tyrosine using molecular oxygen and the cofactor tetrahydrobiopterin (BH4). The enzyme reaction is: (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine. It is vital for producing neurotransmitters and melanin. PAH acts primarily as a homotetramer (dimer of dimers). Each 50-kDa monomer consists of three distinct domains: the N-terminal Regulatory Domain manages allosteric regulation; the Catalytic Domain contains a non-heme iron atom, where the actual conversion of phenylalanine to tyrosine occurs; and the C-terminal Oligomerization Domain facilitates the assembly of monomers into dimers and tetramers. Its activity is regulated by its substrate, phenylalanine, and phosphorylation. PAH is responsible for processing dietary phenylalanine, and its deficiency causes phenylketonuria (PKU), leading to neurotoxic phenylalanine buildup and deficiency in tyrosine-derived neurotransmitters like dopamine and norepinephrine. It is primarily expressed in the liver (hepatocytes), with some evidence of activity in the kidneys. This description may include information annotated by UniProt and/or Google AI.