{"product_id":"anti-dlat-antibody-picoband-r","title":"Anti-DLAT Antibody Picoband®","description":"\u003cp\u003e\u003cb\u003eSize\u003c\/b\u003e: 100 μg\u003c\/p\u003e\u003cp\u003e\u003cb\u003eStorage\u003c\/b\u003e: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eForm\u003c\/b\u003e: Lyophilized\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReactivity\u003c\/b\u003e: Human,Mouse,Rat\u003c\/p\u003e\u003cp\u003e\u003cb\u003eApplications\u003c\/b\u003e: WB,IHC,ICC,IF,Flow Cytometry,ELISA\u003c\/p\u003e\u003cp\u003e\u003cb\u003eApplication Details\u003c\/b\u003e: Western blot, 0.25-0.5 μg\/ml, Human, Mouse, Rat\u003cbr\u003e \nImmunohistochemistry(Paraffin-embedded Section), 2-5 μg\/ml, Human, Mouse, Rat\u003cbr\u003e \nImmunocytochemistry\/Immunofluorescence, 5 μg\/ml, Human\u003cbr\u003e \nFlow Cytometry (Fixed), 1-3 μg\/1x10\u003csup\u003e6\u003c\/sup\u003e cells, Human\u003cbr\u003e \nELISA, 0.1-0.5 μg\/ml, -\u003cbr\u003e\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene Name\u003c\/b\u003e: DLAT\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSpecificity\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e: Dihydrolipoyl transacetylase (or dihydrolipoamide acetyltransferase) is an enzyme component of the multienzyme pyruvate dehydrogenase complex. This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen\u003c\/b\u003e: E.coli-derived human DLAT recombinant protein (Position: P69-P642).\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClonality\u003c\/b\u003e: Polyclonal\u003c\/p\u003e\u003cp\u003e\u003cb\u003eContents\u003c\/b\u003e: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePurification\u003c\/b\u003e: Immunogen affinity purified.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReconstitution\u003c\/b\u003e: Adding 0.2 ml of distilled water will yield a concentration of 500 μg\/ml.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReference\u003c\/b\u003e: 1. Coppel, R. L., McNeilage, L. J., Surh, C. D., Van de Water, J., Spithill, T. W., Whittingham, S., Gershwin, M. E. Primary structure of the human M2 mitochondrial autoantigen of primary biliary cirrhosis: dihydrolipoamide acetyltransferase. Proc. Nat. Acad. Sci. 85: 7317-7321, 1988.\n2. Friedman, J., Feigenbaum, A., Chuang, N., Silhavy, J., Gleeson, J. G. Pyruvate dehydrogenase complex-E2 deficiency causes paroxysmal exercise-induced dyskinesia. Neurology 89: 2297-2298, 2017. \n3. Head, R. A., Brown, R. M., Zolkipli, Z., Shahdadpuri, R., King, M. D., Clayton, P. T., Brown, G. K. Clinical and genetic spectrum of pyruvate dehydrogenase deficiency: dihydrolipoamide acetyltransferase (E2) deficiency. Ann. Neurol. 58: 234-241, 2005.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eUniprot ID\u003c\/b\u003e: P10515\u003c\/p\u003e\u003cp\u003e\u003cb\u003eHost\u003c\/b\u003e: Rabbit\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConcentration\u003c\/b\u003e: Adding 0.2 ml of distilled water will yield a concentration of 500 μg\/ml.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConjugate\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eCross Reactivity\u003c\/b\u003e: No cross-reactivity with other proteins.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eIsotype\u003c\/b\u003e: Rabbit IgG\u003c\/p\u003e\u003cp\u003e\u003cb\u003ePhospho_site\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eClone Number\u003c\/b\u003e: \u003c\/p\u003e\u003cp\u003e\u003cb\u003eObserved Molecular Weight\u003c\/b\u003e: 69 kDa\u003c\/p\u003e\u003cp\u003e\u003cb\u003eCalculated Molecular Weight\u003c\/b\u003e: 128770 MW\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene ID\u003c\/b\u003e: 1737\u003c\/p\u003e\u003cp\u003e\u003cb\u003eProtein Name\u003c\/b\u003e: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial\u003c\/p\u003e\u003cp\u003e\u003cb\u003eGene Full Name\u003c\/b\u003e: dihydrolipoamide S-acetyltransferase\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSynonyms\u003c\/b\u003e: DLAT; DLTA; PBC; PDC E2; PDCE2\u003c\/p\u003e","brand":"GeneBio Systems","offers":[{"title":"Default Title","offer_id":48787707854948,"sku":"A04469-2","price":506.95,"currency_code":"EUR","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0558\/8588\/9636\/files\/no_image_default_image-jpeg_f5060216-ce1c-47b2-bdff-6e989cc612ae.jpg?v=1784402735","url":"https:\/\/www.genebiosystems.com\/en-fr\/products\/anti-dlat-antibody-picoband-r","provider":"GeneBio ","version":"1.0","type":"link"}