Molecular weight (DA)
51kD
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.
Dilution rate
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/40000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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
Protein name
Phenylalanine-4-hydroxylase
Other name
PAH; Phenylalanine-4-hydroxylase; PAH; Phe-4-monooxygenase
Fields
>>Phenylalanine metabolism;>>Phenylalanine, tyrosine and tryptophan biosynthesis;>>Folate biosynthesis;>>Metabolic pathways;>>Biosynthesis of amino acids
Human protein sequence Database
P00439
Mouse protein sequence database
P16331
Rat protein sequence database
P04176
Cellular localization
cytosol,extracellular exosome,
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