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PAH (R400) pAb

PAH (R400) pAb

Cat no: BS3704

Supplier: Bioworld Technology, Inc.
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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.
Catalogue number: BS3704
Reactivities: Human, Mouse, Rat
Hosts: Rabbit
Applications: Immunohistochemistry, Western Blot
Size: 100ug/100ul
Swiss prot: P00439
Dilutions: WB: 1:500~1:1000 IHC: 1:50~1:200
Form: liquid
Storage buffer: 1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.
Concentration: 1ug/ul
Antigen: Synthetic peptide, corresponding to amino acids 371-420 of Human PAH.
Species: PAH (R400) pAb detects endogenous levels of PAH protein.
Molecular weight: ~ 55 kDa
Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage temp: Store at 4 degrees C short term. Aliquot and store at -20 degrees C long term. Avoid freeze-thaw cycles.
Alternative names: Phenylalanine-4-hydroxylase; PAH; Phe-4-monooxygenase
Additional info: For research use only, not for use in diagnostic procedure.

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