PAH Rabbit Polyclonal Antibody

SKU AP20792PU-N

PAH rabbit polyclonal antibody, Aff - Purified

Product Data

• Application: IHC, WB
• Recommended Dilution: Western blot: 1/500 - 1/1000.; Immunohistochemistry on paraffin sections: 1/50 - 1/200.
• Reactivity: Human, Mouse, Rat
• Antibody Host: Rabbit
• Clonality: Polyclonal
• Specificity: This antibody detects endogenous levels of PAH protein.; (region surrounding Arg400)
• Buffer: Phosphate buffered saline (PBS), pH 7.2.; State: Aff - Purified; State: Liquid purified Ig fraction; Preservative: 0.05% sodium azide
• Concentration: 1.0 mg/ml
• Purification: Affinity chromatography (> 95% (by SDS-PAGE)
• Conjugation: Unconjugated
• Storage: Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer.; Avoid repeated freezing and thawing.
• Stability: Shelf life: one year from despatch.
• Shipping: Blue Ice
• Predicted Protein Size: ~ 55 kDa
• Gene Name: phenylalanine hydroxylase
• Database Link:
  P00439
• 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. Numerous mutations can impair the PAH gene, which result in decreased enzyme activity and give rise to varying degrees of PKU. Multiple isozymes of PAH have been reported to exist, but these are most likely allelic variants of PAH that produce protein subunits with slightly different charge and electrophoretic migration.
• Synonyms: Phenylalanine Hydroxylase, PH, PKU1, PKU

Reference Data

• Protein Families: Druggable Genome
• Protein Pathways: Metabolic pathways, Phenylalanine, Phenylalanine metabolism, tyrosine and tryptophan biosynthesis