{"product_id":"cox6b1-antibody-hrp-conjugated-cat-csb-pa00395b0rb","title":"COX6B1 Antibody, HRP conjugated - Cat. #: CSB-PA00395B0Rb","description":"\u003cp\u003e\u003cb\u003eSize \u003c\/b\u003e:50ug\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClone Number\u003c\/b\u003e:\u003c\/p\u003e\u003cp\u003e\u003cb\u003eAliases\u003c\/b\u003e:COX 6B antibody; COX VIb 1 antibody; COX VIb-1 antibody; COX6B antibody; COX6B1 antibody; COXG antibody; COXVIb1 antibody; CX6B1_HUMAN antibody; Cytochrome c oxidase subunit 6B1 antibody; Cytochrome c oxidase subunit VIb antibody; Cytochrome c oxidase subunit VIb isoform 1 antibody; cytochrome c oxidase subunit VIb polypeptide 1 (ubiquitous) antibody; Cytochrome c oxidase subunit Vib polypeptide 1 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:P14854\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen\u003c\/b\u003e:Recombinant Human Cytochrome c oxidase subunit 6B1 protein (1-86AA)\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\u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e:Defects in COX6B1 are a cause of mitochondrial complex IV deficiency (MT-C4D) [MIM:220110]; also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome.\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:\u0026gt;95%, Protein G purified\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConjugate\u003c\/b\u003e:HRP\u003c\/p\u003e\u003cp\u003e\u003cb\u003eBuffer\u003c\/b\u003e:Preservative: 0.03% Proclin 300\u003cbr\u003eConstituents: 50% Glycerol, 0.01M PBS, PH 7.4\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:COX6B1\u003c\/p\u003e\u003cp\u003e\u003cb\u003eResearch Areas\u003c\/b\u003e:Cancer; Tags \u0026amp; Cell Markers; Metabolism; Signal transduction\u003c\/p\u003e","brand":"Gene Bio Systems","offers":[{"title":"Default Title","offer_id":40484096376932,"sku":"CSB-PA00395B0Rb","price":61600.0,"currency_code":"JPY","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0558\/8588\/9636\/products\/no_image_default_image-jpeg_3c51d2c3-bcda-4622-a47a-2fd45702a4b7.jpg?v=1660088345","url":"https:\/\/www.genebiosystems.com\/en-jp\/products\/cox6b1-antibody-hrp-conjugated-cat-csb-pa00395b0rb","provider":"GeneBio ","version":"1.0","type":"link"}