{"product_id":"ndufb8-recombinant-rabbit-mab-s-4770-29-s0b60407","title":"NDUFB8 Recombinant Rabbit mAb (S-4770-29)","description":"\u003ch4\u003eProduct Specification\u003c\/h4\u003e\u003cdiv class=\"responsive-table product-detail-table details-table\"\u003e\n\u003cbr\u003e\u003ctable style=\"width: 100%; height: auto;\"\u003e\u003ctbody\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eHost\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eRabbit\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eAntigen\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eNDUFB8\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eSynonyms\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eNADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial; Complex I-ASHI (CI-ASHI); NADH-ubiquinone oxidoreductase ASHI subunit\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eImmunogen\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eRecombinant Protein\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eLocation\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eMitochondrion\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eAccession\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eO95169\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eClone Number\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eS-4770-29\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eAntibody Type\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eRecombinant mAb\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eIsotype\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eIgG\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eApplication\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eWB, IHC-P\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eReactivity\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eHu, Ms, Rt\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003ePositive Sample\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eHCT116, HeLa, A549, 293T, SW480, HepG2, mouse brain, mouse heart, mouse kidney, rat brain, rat heart, rat kidney\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003ePurification\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eProtein A\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eConcentration\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003e0.5 mg\/ml\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eConjugation\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eUnconjugated\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003ePhysical Appearance\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eLiquid\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eStorage Buffer\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003e\u003cp\u003ePBS, 40% Glycerol, 0.05% BSA, 0.02% sodium azide\u003c\/p\u003e\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003eStability \u0026amp; Storage\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003e\u003cp\u003e12 months from date of receipt \/ reconstitution, -20 °C as supplied\u003c\/p\u003e\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003c\/tbody\u003e\u003c\/table\u003e\n\u003c\/div\u003e\u003ch4\u003eDilution\u003c\/h4\u003e\u003cdiv class=\"responsive-table product-detail-table details-table\"\u003e\n\u003cbr\u003e\u003ctable style=\"width: 60%; height: auto;\"\u003e\u003ctbody\u003e\n\u003ctr\u003e\n\u003ctd\u003e\u003cstrong\u003eapplication\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd\u003e\u003cstrong\u003edilution\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd\u003e\u003cstrong\u003especies\u003c\/strong\u003e\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd\u003eWB\u003c\/td\u003e\n\u003ctd\u003e1:1000-1:2000\u003c\/td\u003e\n\u003ctd\u003eHu, Ms, Rt\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd\u003eIHC-P\u003c\/td\u003e\n\u003ctd\u003e1:100-1:500\u003c\/td\u003e\n\u003ctd\u003eHu, Ms, Rt\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003c\/tbody\u003e\u003c\/table\u003e\n\u003c\/div\u003e\u003ch4\u003eBackground\u003c\/h4\u003e\u003cdiv\u003e\u003cp\u003e\u003cspan\u003eNDUFB8, namely NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, is a critical accessory subunit of mitochondrial respiratory chain Complex I (NADH:ubiquinone oxidoreductase). Although it does not directly participate in catalysis, it is essential for the stable assembly and normal function of Complex I, with its specific role being to stabilize the structural module where ubiquinone reduction occurs and to support the proton translocation pathway. This protein is located in the inner mitochondrial membrane. Biallelic loss-of-function mutations in its encoding gene, NDUFB8, lead to isolated Complex I deficiency, resulting in an autosomal recessive primary mitochondrial disease (namely nuclear-type mitochondrial Complex I deficiency type 32). Patients typically present with infantile-onset encephalomyopathy resembling Leigh syndrome, characterized by developmental delay, seizures, hypotonia, and lactic acidosis. In addition, abnormal expression or functional alterations of NDUFB8 are also associated with neurodegenerative diseases such as Alzheimer's disease and Parkinson's disease, and may serve as potential biomarkers for these conditions.\u003c\/span\u003e\u003c\/p\u003e\u003c\/div\u003e","brand":"Starter","offers":[{"title":"25μl","offer_id":43103726534731,"sku":"S0B60407-25μl","price":100.0,"currency_code":"USD","in_stock":true},{"title":"100μl","offer_id":43103726567499,"sku":"S0B60407-100μl","price":350.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/8375\/1499\/files\/eafeead4ca704c06ad91cd50b3f21d09.jpg?v=1788292948","url":"https:\/\/www.antbioinc.com\/products\/ndufb8-recombinant-rabbit-mab-s-4770-29-s0b60407","provider":"AntBio","version":"1.0","type":"link"}