PAH (R400) polyclonal Antibody | BS3704

PAH (R400) polyclonal Antibody | BS3704 | Gentaur UK, US & Europe Distribution

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: