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PAH (R400) polyclonal antibody

货号: BS3704
价格 1140
货期 现货(1-2个工作日)
25ul 50ul 100ul 200ul
No. :BS3704
Product Name :PAH (R400) polyclonal antibody
Swiss-Prot :P00439
Host :Rabbit
Reactivity :Human,Mouse,Rat
Applications :IHC
Application_all :IHC:1:50~1:200
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.
Product :Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Purification&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&Stability :Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Specificity :PAH (R400) polyclonal antibody detects endogenous levels of PAH protein.
BiowMW :~ 52 kDa
Note :For research use only, not for use in diagnostic procedure.
Alternative Name :Phenylalanine-4-hydroxylase; PAH; Phe-4-monooxygenase
Immunogen :Synthetic peptide, corresponding to amino acids 371-420 of Human PAH.
Conjugate :Unconjugated
Modification :Unmodification
  • A novel Pah-exon1 deleted murine model of phenylalanine hydroxylase (PAH) deficiency
    JournalMolecular Genetics And Metabolism
    ApplicationWB,IHC
    IF4.797
    SpeciesMouse
  • Development of a porcine model of phenylketonuria with a humanized R408W mutation for gene editing
    JournalPLoS One
    ApplicationWB
    IF3.24
    SpeciesPig

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400-660-9091

电话:025-68037686

地址:江苏生命科技创新园F6幢1层

订购:nanjing03@biogot.com

服务:biorase01@biogot.com

合作:lvyun@biogot.com

支持:may@biogot.com