Specifications
Species Reactivity
Mouse; Rat; Human
Immunogen
Synthetic peptide (the amino acid sequence is considered to be commercially sensitive) within Human PAH aa 50-150 (Cysteine residue). The exact sequence is proprietary.Database link: P00439
Applications
Application Notes
WB: 1/1000 - 1/10000; IHC-P: 1/50 - 1/100; ICC/IF: 1/50 - 1/100; Flow Cyt: 1/10 - 1/100;
Target
Alternative Names
PAH; phenylalanine hydroxylase; phenylalanine-4-hydroxylase; PH; phenylalanine 4 monooxygenase; phe-4-monooxygenase; phenylalanine 4-monooxygenase; PKU; PKU1;
Product Background
Antigen 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.
Pathway
Biogenic Amine Synthesis, organism-specific biosystem; Metabolic pathways, organism-specific biosystem; Metabolism, organism-specific biosystem; Metabolism of amino acids and derivatives, organism-specific biosystem; Phenylalanine and tyrosine catabolism, organism-specific biosystem; Phenylalanine metabolism, organism-specific biosystem; Phenylalanine metabolism, conserved biosystem;
Citations
Publication ()
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COA
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