{"product_id":"gch1-antibody-cat-csb-pa964562","title":"GCH1 Antibody - Cat. #: CSB-PA964562","description":"\u003cp\u003e\u003cb\u003eSize \u003c\/b\u003e:50ul\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClone Number\u003c\/b\u003e:\u003c\/p\u003e\u003cp\u003e\u003cb\u003eAliases\u003c\/b\u003e:dystonia 14 antibody; DYT 5 antibody; DYT14 antibody; DYT5 antibody; DYT5a antibody; GCH 1 antibody; GCH antibody; Gch1 antibody; GCH1_HUMAN antibody; GTP CH 1 antibody; GTP CH I antibody; GTP cyclohydrolase 1 (dopa responsive dystonia) antibody; GTP cyclohydrolase 1 antibody; GTP cyclohydrolase I antibody; GTP-CH-I antibody; GTPCH 1 antibody; GTPCH1 antibody; Guanosine 5' triphosphate cyclohydrolase I antibody; HPABH4B antibody\u003c\/p\u003e\u003cp\u003e\u003cb\u003eProduct Type\u003c\/b\u003e:Polyclonal Antibody\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen Species\u003c\/b\u003e:Homo sapiens (Human)\u003c\/p\u003e\u003cp\u003e\u003cb\u003eUniProt ID\u003c\/b\u003e:P30793\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen\u003c\/b\u003e:Synthetic peptide of Human GCH1\u003c\/p\u003e\u003cp\u003e\u003cb\u003eRaised in\u003c\/b\u003e:Rabbit\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSpecies Reactivity\u003c\/b\u003e:Human\u003c\/p\u003e\u003cp\u003e\u003cb\u003eTested Applications\u003c\/b\u003e:ELISA, WB, IHC; ELISA:1:1000-1:5000, WB:1:500-1:2000, IHC:1:5-1:20\u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e:This gene encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme in tetrahydrobiopterin (BH4) biosynthesis, catalyzing the conversion of GTP into 7,8-dihydroneopterin triphosphate. BH4 is an essential cofactor required by aromatic amino acid hydroxylases as well as nitric oxide synthases. Mutations in this gene are associated with malignant hyperphenylalaninemia and dopa-responsive dystonia. Several alternatively spliced transcript variants encoding different isoforms have been described; however, not all variants give rise to a functional enzyme.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClonality\u003c\/b\u003e:Polyclonal\u003c\/p\u003e\u003cp\u003e\u003cb\u003eIsotype\u003c\/b\u003e:IgG\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePurification Method\u003c\/b\u003e:Antigen affinity purification\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConjugate\u003c\/b\u003e:Non-conjugated\u003c\/p\u003e\u003cp\u003e\u003cb\u003eBuffer\u003c\/b\u003e:-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol\u003c\/p\u003e\u003cp\u003e\u003cb\u003eForm\u003c\/b\u003e:Liquid\u003c\/p\u003e\u003cp\u003e\u003cb\u003eStroage\u003c\/b\u003e:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eTarget Names\u003c\/b\u003e:GCH1\u003c\/p\u003e\u003cp\u003e\u003cb\u003eResearch Areas\u003c\/b\u003e:Neuroscience?Cancer;Cell biology;Metabolism;Signal transduction\u003c\/p\u003e","brand":"Gene Bio Systems","offers":[{"title":"Default Title","offer_id":40483448356964,"sku":"CSB-PA964562","price":484.78,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0558\/8588\/9636\/products\/no_image_default_image-jpeg_8074c6fa-247e-421a-86ab-12572ccfaf7c.jpg?v=1660069172","url":"https:\/\/www.genebiosystems.com\/products\/gch1-antibody-cat-csb-pa964562","provider":"GeneBio ","version":"1.0","type":"link"}