Target：PAH

Fields：Phenylalanine metabolism;Phenylalanine, tyrosine and tryptophan biosynthesis;Folate biosynthesis;Metabolic pathways;Biosynthesis of amino acids

Gene Name：PAH

Protein Name：Phenylalanine-4-hydroxylase

Human Gene Id：5053

Human Swiss Prot No：P00439

Mouse Gene Id：18478

Mouse Swiss Prot No：P16331

Rat Swiss Prot No：P04176

Immunogen：The antiserum was produced against synthesized peptide derived from human PAH. AA range:351-400

Specificity：PAH Polyclonal Antibody detects endogenous levels of PAH protein.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：PAH;Phenylalanine-4-hydroxylase;PAH;Phe-4-monooxygenase

Observed Band(KD)：51kD

Background： 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],

Function：catalytic activity:L-phenylalanine + tetrahydrobiopterin + O(2) = L-tyrosine + 4a-hydroxytetrahydrobiopterin.,cofactor:Fe(2+) ion.,disease:Defects in PAH are the cause of hyperphenylalaninemia (HPA) [MIM:261600]. HPA is the mildest form of phenylalanine hydroxylase deficiency.,disease: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.,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 hydroxylas

Subcellular Location：cytosol,extracellular exosome,

Expression： Liver,