{"product_id":"pah-r400-polyclonal-antibody-bs3704","title":"PAH (R400) Polyclonal Antibody-BS3704","description":"\u003cp\u003ePAH (R400) Polyclonal Antibody\u003c\/p\u003e\n\n\u003cp\u003eProduct: Rabbit IgG, 1mg\/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2\u003c\/p\u003e\n\n\u003cp\u003eCatalogue Numbers: BS3704-50, BS3704-100\u003c\/p\u003e\n\n\u003cp\u003eSizes: 50µl, 100µl\u003c\/p\u003e\n\n\u003cp\u003eSwiss-Prot: P00439\u003c\/p\u003e\n\n\u003cp\u003eHost: Rabbit\u003c\/p\u003e\n\n\u003cp\u003eReactivity: Human, Mouse, Rat\u003c\/p\u003e\n\n\u003cp\u003eApplications: IHC\u003c\/p\u003e\n\n\u003cp\u003eBackground: 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.\u003c\/p\u003e \n\n\u003cp\u003eProduct: Rabbit IgG, 1mg\/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2\u003c\/p\u003e\n\n\u003cp\u003ePurification and Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is \u0026gt; 95% (by SDS-PAGE).\u003c\/p\u003e\n\n\u003cp\u003eSpecificity: PAH (R400) polyclonal antibody detects endogenous levels of PAH protein.\u003c\/p\u003e\n\n\u003cp\u003eNote: For research use only, not for use in diagnostic procedure.\u003c\/p\u003e\n\n\u003cp\u003eExtra Notes: Immunohistochemistry (IHC) analyzes of PAH (R400) pAb in paraffin-embedded human brain tissue.\u003c\/p\u003e \n\n\u003cp\u003eBioworld Molecular Weight: ~ 52 kDa\u003c\/p\u003e\n\n\u003cp\u003eStorage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.\u003c\/p\u003e\n\n\u003cp\u003eAlternative Name: Phenylalanine-4-hydroxylase; PAH; Phe-4-monooxygenase\u003c\/p\u003e\n\n\u003cp\u003eImmunogen: Synthetic peptide, corresponding to amino acids 371-420 of Human PAH.\u003c\/p\u003e\n\n\u003cp\u003eConjugate: Unconjugated\u003c\/p\u003e\n\n\u003cp\u003eModification: Unmodified\u003c\/p\u003e","brand":"BioWorld","offers":[{"title":"50µl","offer_id":42949468291251,"sku":"BS3704-50","price":254.38,"currency_code":"CAD","in_stock":true},{"title":"100µl","offer_id":42949468324019,"sku":"BS3704-100","price":442.75,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0305\/9482\/6376\/products\/thumbnail_d1447bac803ec9be_11-14-09-19-51_ba29e539-e0da-4a0f-81a1-292db85d3e3f.jpg?v=1704970050","url":"https:\/\/afsbio.com\/products\/pah-r400-polyclonal-antibody-bs3704","provider":"AFSBio Inc.","version":"1.0","type":"link"}