{"product_id":"ndufa9-recombinant-mouse-mab-s-5382-s0b60187","title":"NDUFA9 Recombinant Mouse mAb (S-5382)","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%;\"\u003eMouse\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%;\"\u003eNDUFA9\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 alpha subcomplex subunit 9, mitochondrial; Complex I-39kD (CI-39kD); NADH-ubiquinone oxidoreductase 39 kDa subunit; NDUFS2L\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%;\"\u003eQ16795\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-5382\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%;\"\u003eIgG1,k\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%;\"\u003eWI-38, mouse heart, rat heart\u003c\/td\u003e\n\u003c\/tr\u003e\n\u003ctr\u003e\n\u003ctd style=\"width: 22%;\"\u003e\u003cstrong\u003ePredicted Reactivity\u003c\/strong\u003e\u003c\/td\u003e\n\u003ctd style=\"width: 78%;\"\u003eBv\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 G\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%;\"\u003e1.5 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\u003e1:1000-1:10000\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\u003e1:500\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\u003eNDUFA9 is a mitochondrial protein encoded by the NDUFA9 gene, functioning as a 39-kDa non-catalytic accessory subunit of respiratory chain complex I. After being synthesized in the cytoplasm, this protein is transported to the mitochondrial matrix and is primarily localized to the Q module of complex I, where it plays a critical role in maintaining the structural stability and normal function of complex I. Although NDUFA9 is not directly involved in the catalytic electron transfer reactions, it is essential for the proper assembly of complex I and the stabilization of late assembly intermediates, and it may also participate in recruiting the substrate ubiquinone-10. At the pathological level, homozygous or compound heterozygous mutations in the NDUFA9 gene can lead to nuclear type 26 mitochondrial complex I deficiency (MC1DN26), a disorder inherited in an autosomal recessive manner with a broad clinical spectrum, ranging from neonatal-lethal Leigh syndrome to childhood-onset progressive neurodevelopmental disorders with prominent dystonia. Affected patients typically present with developmental delay, dystonia, seizures, and abnormal MRI signals in the basal ganglia, and the underlying pathogenesis lies in the failure of complex I assembly and a significant reduction in its activity caused by these mutations.\u003c\/span\u003e\u003c\/p\u003e\u003c\/div\u003e","brand":"Starter","offers":[{"title":"25μl","offer_id":43096736694347,"sku":"S0B60187-25μl","price":100.0,"currency_code":"USD","in_stock":true},{"title":"100μl","offer_id":43096736727115,"sku":"S0B60187-100μl","price":350.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/8375\/1499\/files\/66eba9d2ceed47b196f95035b0b8c30a.jpg?v=1788242566","url":"https:\/\/www.antbioinc.com\/products\/ndufa9-recombinant-mouse-mab-s-5382-s0b60187","provider":"AntBio","version":"1.0","type":"link"}