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PAH (R400) Polyclonal Antibody-BS3704
PAH (R400) Polyclonal Antibody-BS3704
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PAH (R400) Polyclonal Antibody
Product: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Catalogue Numbers: BS3704-50, BS3704-100
Sizes: 50µl, 100µl
Swiss-Prot: P00439
Host: Rabbit
Reactivity: Human, Mouse, Rat
Applications: IHC
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.
Product: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Purification and Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Specificity: PAH (R400) polyclonal antibody detects endogenous levels of PAH protein.
Note: For research use only, not for use in diagnostic procedure.
Extra Notes: Immunohistochemistry (IHC) analyzes of PAH (R400) pAb in paraffin-embedded human brain tissue.
Bioworld Molecular Weight: ~ 52 kDa
Storage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Alternative Name: Phenylalanine-4-hydroxylase; PAH; Phe-4-monooxygenase
Immunogen: Synthetic peptide, corresponding to amino acids 371-420 of Human PAH.
Conjugate: Unconjugated
Modification: Unmodified