• 靶点：
• PAH

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

• 基因名称：
• PAH

• 蛋白名称：
• 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

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

• 特异性：
• PAH Polyclonal Antibody detects endogenous levels of PAH protein.

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

• 来源：
• Polyclonal, Rabbit,IgG

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

• 纯化工艺：
• The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

• 浓度：
• 1 mg/ml

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

• 其他名称：
• PAH;Phenylalanine-4-hydroxylase;PAH;Phe-4-monooxygenase

• 实测条带：
• 51kD

• 背景：
• 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],

• 功能：
• 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

• 细胞定位：
• cytosol,extracellular exosome,

• 组织表达：
• Liver,

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