{"product_id":"fh-antibody-cat-csb-pa045962","title":"FH Antibody - Cat. #: CSB-PA045962","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:FH antibody; Fumarase antibody; Fumarate hydratase antibody; Fumarate hydratase mitochondrial antibody; Fumarate hydratase; mitochondrial antibody; FUMH_HUMAN antibody; HLRCC antibody; LRCC antibody; MCL antibody; MCUL 1 antibody; MCUL1 antibody; MS709 antibody; Multiple hereditary cutaneous leiomyomata 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:P07954\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen\u003c\/b\u003e:Fusion protein of Human FH\u003c\/p\u003e\u003cp\u003e\u003cb\u003eRaised in\u003c\/b\u003e:Rabbit\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSpecies Reactivity\u003c\/b\u003e:Human, Mouse, Rat\u003c\/p\u003e\u003cp\u003e\u003cb\u003eTested Applications\u003c\/b\u003e:ELISA, WB, IHC; ELISA:1:2000-1:5000, WB:1:500-1:2000, IHC:1:50-1:200\u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e:The protein encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy.\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:FH\u003c\/p\u003e\u003cp\u003e\u003cb\u003eResearch Areas\u003c\/b\u003e:Cancer;Metabolism;Signal transduction\u003c\/p\u003e","brand":"Gene Bio Systems","offers":[{"title":"Default Title","offer_id":40482907324516,"sku":"CSB-PA045962","price":61500.0,"currency_code":"JPY","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0558\/8588\/9636\/products\/no_image_default_image-jpeg_3875a493-c342-4724-b6c2-07308005b35c.jpg?v=1660059253","url":"https:\/\/www.genebiosystems.com\/en-jp\/products\/fh-antibody-cat-csb-pa045962","provider":"GeneBio ","version":"1.0","type":"link"}