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
Curated Selection
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