Product Description

PAH (R400) Polyclonal Antibody

Sizes: 50μl, 100μl

Catalogue Numbers: BS3704-50, BS3704-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

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 resμlts 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.

Category: Primary Antibody

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: IHC

Alternate Names: Phenylalanine-4-hydroxylase, PAH, Phe-4-monooxygenase

Clonality: Polyclonal

Conjugate: Unconjugated

Modification: Unmodification

Immunogen: Synthetic peptide, corresponding to amino acids 371-420 of Human PAH.

Dilution: IHC:1:50~1:200

Purification: 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.

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.

SwissProt: P00439

Product: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Research Use Only