{"product_id":"dlat-rabbit-pab-a6288","title":"DLAT Rabbit pAb - A6288","description":"\u003cp\u003eDLAT Rabbit pAb\u003c\/p\u003e\n\n\u003cp\u003eSizes: 20μL, 100μL\u003c\/p\u003e\n\n\u003cp\u003eCatalogue Numbers: A6288-20, A6288-100\u003c\/p\u003e\n\n\u003cp\u003eCitations, Manuals and MSDS Available upon request.\u003c\/p\u003e\n\n\u003cp\u003eBackground: 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\n\n\u003cp\u003eCategory: Polyclonal Antibodies\u003c\/p\u003e\n\n\u003cp\u003eApplications: WB, IF\/ICC, ELISA\u003c\/p\u003e\n\n\u003cp\u003eCross-reactivity: Human, Mouse, Rat\u003c\/p\u003e\n\n\u003cp\u003eProtein Weight: 69kDa\u003c\/p\u003e\n\n\u003cp\u003eObserved Molecular Weight: 69kDa\u003c\/p\u003e\n\n\u003cp\u003eImmunogen: Recombinant protein (or fragment) .\u003c\/p\u003e\n\n\u003cp\u003eSpecies: Human\u003c\/p\u003e\n\n\u003cp\u003eGeneID: 1737\u003c\/p\u003e\n\n\u003cp\u003eSWISS: P10515\u003c\/p\u003e\n\n\u003cp\u003eAlternate Names: E2; PBC; DLTA; PDCE2; PDC-E2; DLAT\u003c\/p\u003e\n\n\u003cp\u003eSource: Rabbit\u003c\/p\u003e\n\n\u003cp\u003eIsotype: IgG\u003c\/p\u003e\n\n\u003cp\u003ePurity: Affinity purification\u003c\/p\u003e\n\n\u003cp\u003eStorage: Store at -20℃. Avoid freeze \/ thaw cycles. Buffer: PBS with 0.09% Sodium azide, 50% glycerol, pH 7.3.\u003c\/p\u003e\n\n\u003cp\u003eRecommended Dilutions: WB, 1:500 - 1:2000 IF\/ICC, 1:20 - 1:50 ELISA, Recommended starting concentration is 1 μg\/mL. Please optimize the concentration based on your specific assay requirements.\u003c\/p\u003e\n\n\u003cp\u003eResearch Areas: Cancer, Signal Transduction, Endocrine Metabolism, Mitochondrial metabolism, Mitochondrial markers, Carbohydrate metabolism.\u003c\/p\u003e\n\n\u003cp\u003eNCBI Alias: DLAT\u003c\/p\u003e\n\n\u003cp\u003eResearch Use Only\u003c\/p\u003e","brand":"ABclonal","offers":[{"title":"20uL","offer_id":45189982355635,"sku":"A6288-20","price":151.2,"currency_code":"CAD","in_stock":true},{"title":"100uL","offer_id":45189982388403,"sku":"A6288-100","price":417.2,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0305\/9482\/6376\/files\/download_ecb4fc15-1397-43fa-a4f8-95105d990e01.png?v=1766439240","url":"https:\/\/afsbio.com\/products\/dlat-rabbit-pab-a6288","provider":"AFSBio Inc.","version":"1.0","type":"link"}