{"product_id":"new-product-390315","title":"ATP6 Rabbit Polyclonal Antibody","description":"\u003cp\u003eATP6 Rabbit Polyclonal Antibody\u003c\/p\u003e\n\u003cdiv class=\"item item1\"\u003e\n\u003ch4 class=\"tit clearfix tabs\"\u003e\u003cspan class=\"fz20 fl tran300 active\"\u003eOverview\u003c\/span\u003e\u003c\/h4\u003e\n\u003cdiv class=\"cont fz16 pageStyle\"\u003e\n\u003cdiv id=\"tabs-container\" class=\"container1 swiper-container-horizontal swiper-container-autoheight\"\u003e\n\u003cdiv class=\"swiper-wrapper\"\u003e\n\u003cdiv class=\"swiper-slide pageStyle fz16 swiper-no-swiping swiper-slide-active\"\u003e\n\u003ctable class=\"table2\"\u003e\n\u003ctbody\u003e\n\u003ctr\u003e\n\u003ctd width=\"208px\" class=\"fz18\"\u003eProduct name:\u003c\/td\u003e\n\u003ctd\u003eATP6 rabbit pAb\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eApplications:\u003c\/td\u003e\n\u003ctd\u003eWB;ELISA\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eRecommended Dilutions:\u003c\/td\u003e\n\u003ctd\u003eWB 1:500-2000 ELISA 1:5000-20000\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eImmunogen:\u003c\/td\u003e\n\u003ctd\u003eSynthesized peptide derived from human protein . at AA range: 60-140\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eStorage:\u003c\/td\u003e\n\u003ctd\u003eRabbit\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eStorage:\u003c\/td\u003e\n\u003ctd\u003e-20°C\/1 year\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eClonality:\u003c\/td\u003e\n\u003ctd\u003ePolyclonal\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eIsotype:\u003c\/td\u003e\n\u003ctd\u003eIgG\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eConcentration:\u003c\/td\u003e\n\u003ctd\u003e1 mg\/ml\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eObserved Band:\u003c\/td\u003e\n\u003ctd\u003e24kD\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eGeneID（Human）:\u003c\/td\u003e\n\u003ctd\u003e4508\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eHuman Swiss-Prot No:\u003c\/td\u003e\n\u003ctd\u003eP00846\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eCellular localization:\u003c\/td\u003e\n\u003ctd\u003eMitochondrion inner membrane; Multi-pass membrane protein.\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eBackground:\u003c\/td\u003e\n\u003ctd\u003edisease:Defects in MT-ATP6 are a cause of infantile bilateral striatal necrosis [MIM:500003]. Bilateral striatal necrosis is a neurological disorder resembling Leigh syndrome.,disease:Defects in MT-ATP6 are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.,disease:Defects in MT-ATP6 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.,disease:Defects in MT-ATP6 are the cause of neurogenic muscle weakness, ataxia, and retinitis pigmentosa (NARP) [MIM:551500].,disease:Defects in MT-CO3 are a cause of cytochrome c oxidase deficiency (COX deficiency) [MIM:220110]; also called mitochondrial complex IV deficiency. COX deficiency is a clinically heterogeneous disorder. The clinical features are ranging from isolated myopathy to severe multisystem disease, with onset from infancy to adulthood.,disease:Defects in MT-CO3 are a cause of Leber hereditary optic neuropathy (LHON) [MIM:535000]. LHON is a maternally inherited disease resulting in acute or subacute loss of central vision, due to optic nerve dysfunction. Cardiac conduction defects and neurological defects have also been described in some patients. LHON results from primary mitochondrial DNA mutations affecting the respiratory chain complexes.,disease:Defects in MT-CO3 are associated with recurrent myoglobinuria [MIM:550500]. Myoglobinuria consists of excretion of myoglobin in the urine.,disease:Defects in MT-CO3 are found in mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) syndrome, a genetically heterogeneous disorder, characterized by episodic vomiting, seizures, and recurrent cerebral insults resembling strokes and causing hemiparesis, hemianopsia, or cortical blindness.,function:Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the central stalk subunits to proton translocation. Key component of the proton channel; it may play a direct role in the translocation of protons across the membrane.,function:Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the central stalk subunits to proton translocation. Part of the complex F(0) domain. Minor subunit located with subunit a in the membrane.,function:Subunits I, II and III form the functional core of the enzyme complex.,similarity:Belongs to the ATPase A chain family.,similarity:Belongs to the ATPase protein 8 family.,similarity:Belongs to the cytochrome c oxidase subunit 3 family.,subunit:F-type ATPases have 2 components, CF(1) - the catalytic core - and CF(0) - the membrane proton channel.,subunit:F-type ATPases have 2 components, CF(1) - the catalytic core - and CF(0) - the membrane proton channel. CF(1) has five subunits: alpha(3), beta(3), gamma(1), delta(1), epsilon(1). CF(0) has three main subunits: a, b and c.,\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd class=\"fz18\"\u003eSpecies Reactivity:\u003c\/td\u003e\n\u003ctd\u003eHuman;Rat;Mouse;\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003c\/tbody\u003e\n\u003c\/table\u003e\n\u003c\/div\u003e\n\u003c\/div\u003e\n\u003c\/div\u003e\n\u003c\/div\u003e\n\u003c\/div\u003e\n\u003cdiv class=\"item item5\"\u003e\n\u003cul\u003e\n\u003cli\u003e\u003ca class=\"scaleimg\"\u003e\n\u003cdiv class=\"pic\"\u003e\u003cimg class=\"tran500\" alt=\"\" src=\"https:\/\/www.elkbiotech.com\/upload\/image\/Antibodies\/pAb\/ES9414-c-1.jpg\"\u003e\u003c\/div\u003e\n\u003cdiv class=\"text fz16\"\u003eWestern blot analysis of lysates from SW480 cells, primary antibody was diluted at 1:1000, 4°over night\u003c\/div\u003e\n\u003c\/a\u003e\u003c\/li\u003e\n\u003c\/ul\u003e\n\u003c\/div\u003e","brand":"ELK Biotechnology","offers":[{"title":"100ul","offer_id":47447717413144,"sku":"ES9414","price":248.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/products\/ELK_649db345-53c4-4370-8463-ef433a408471.jpg?v=1704339360","url":"https:\/\/danabiosci.com\/products\/new-product-390315","provider":"Dana Bioscience","version":"1.0","type":"link"}