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PAH (R400) polyclonal antibody

Product Specifications

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.

CAS Number

9007-83-4

Structure Composition

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

Product Name Alternative

Phenylalanine-4-hydroxylase; PAH; Phe-4-monooxygenase

Swiss Prot

P00439

Reactivity

Human, Mouse, Rat

Immunogen

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

Conjugation

Unconjugated

Applications

IHC

Dilution

IHC:1:50~1:200

Purity

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .

Modification

Unmodification

Molecular Weight

~ 52 kDa

Storage Conditions

Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

Notes

For research use only, not for use in diagnostic procedure.

Specificity

PAH (R400) polyclonal antibody detects endogenous levels of PAH protein.

Host or Source

Rabbit

Available Sizes

Frequently Asked Questions

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