{"product_id":"human-phenylalanine-4-hydroxylase-pah-elisa-kit","title":"Human Phenylalanine-4-hydroxylase, PAH ELISA Kit","description":"\u003cp\u003e\u003cb\u003eSize\u003c\/b\u003e:96 Tests-1 Kit\u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e:Phenylalanine hydroxylase (PAH) (EC 1.14.16.1) is an enzyme that catalyzes the hydroxylation of the aromatic side-chain of phenylalanine to generate tyrosine. PAH is one of three members of the biopterin-dependent aromatic amino acid hydroxylases, a class of monooxygenase that uses tetrahydrobiopterin (BH4, a pteridine cofactor) and a non-heme iron for catalysis. During the reaction, molecular oxygen is heterolytically cleaved with sequential incorporation of one oxygen atom into BH4 and phenylalanine substrate. In humans, mutations in its encoding gene, PAH, can lead to the metabolic disorder phenylketonuria.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eResearch_area\u003c\/b\u003e:Signal Transduction; Metabolism\u003c\/p\u003e\u003cp\u003e\u003cb\u003eApplications\u003c\/b\u003e:ELISA\u003c\/p\u003e\u003cp\u003e\u003cb\u003eTarget_protein\u003c\/b\u003e:PAH\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReactivity\u003c\/b\u003e:Human\u003c\/p\u003e\u003cp\u003e\u003cb\u003eAssay type\u003c\/b\u003e:Sandwich\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSensitivity\u003c\/b\u003e:4.86ng\/L\u003c\/p\u003e\u003cp\u003e\u003cb\u003eDetection range\u003c\/b\u003e:10-2000ng\/L\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSample type\u003c\/b\u003e:Serum, plasma or other biological fluids\u003c\/p\u003e\u003cp\u003e\u003cb\u003eFull product name\u003c\/b\u003e:Phenylalanine-4-hydroxylase\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSpecies\u003c\/b\u003e:Human\u003c\/p\u003e\u003cp\u003e\u003cb\u003eStorage\u003c\/b\u003e:2-8ºC\u003c\/p\u003e\u003cp\u003e\u003cb\u003eAssay time\u003c\/b\u003e:1h 30m\u003c\/p\u003e\u003cp\u003e\u003cb\u003eUniProt accession\u003c\/b\u003e:P00439\u003c\/p\u003e\u003cp\u003e\u003cb\u003eMASS(Da)\u003c\/b\u003e:51,862\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGeneID\u003c\/b\u003e:5053\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSynonyms\u003c\/b\u003e:PAH;PH;Phe-4-monooxygenase;Phenylalanine-4-hydroxylase;PKU;PKU1\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene names\u003c\/b\u003e:PAH\u003c\/p\u003e\u003cp\u003e\u003cb\u003eStandard solution\u003c\/b\u003e:2400ng\/L\u003c\/p\u003e","brand":"GeneBio Systems","offers":[{"title":"Default Title","offer_id":48648178532452,"sku":"E6875Hu","price":128800.0,"currency_code":"JPY","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0558\/8588\/9636\/files\/no_image_default_image-jpeg_6465dfca-ae37-4b1e-81fe-97747fda66e8.jpg?v=1782711161","url":"https:\/\/www.genebiosystems.com\/en-jp\/products\/human-phenylalanine-4-hydroxylase-pah-elisa-kit","provider":"GeneBio ","version":"1.0","type":"link"}