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Product Description
PAH (R400) polyclonal Antibody | BS3704 | Bioworld
Host: Rabbit
Reactivity: Human,Mouse,Rat
Application: IHC
Application Range: IHC:1:50~1:200
Background: The PAH gene encodes the enzyme phenylalanine hydroxylase (PAH), which converts phenylalanine to tyrosine and is the rate-limiting enzyme in phenylalanine catabolism. Mammalian PAH is a soluble, homotetrameric protein which is abundantly expressed in human liver. Deficiency of PAH activity results in the autosomal recessive disorder phenylketonuria (PKU), which is characterized by mental retardation unless a low phenylalanine diet is introduced early in life. The PAH gene, which maps to human chromosome 12q23.2, contains all the genetic information necessary to code for functional PAH, demonstrating that a single gene is involved in the classic disease phenotype.
Storage & Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Specificity: PAH (R400) polyclonal Antibody detects endogenous levels of PAH protein.
Molecular Weight: ~ 52 kDa
Note: For research use only, not for use in diagnostic procedure.
Alternative Names: Phenylalanine-4-hydroxylase; PAH; Phe-4-monooxygenase
Immunogen: Synthetic peptide, corresponding to amino acids 371-420 of Human PAH.
Conjugate: Unconjugated
Modification: Unmodification
Purification & Purity: The Antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .
Pathway:
