{"product_id":"bckdha-rabbit-pab-a21588","title":"BCKDHA Rabbit pAb - A21588","description":"\u003cp\u003eBCKDHA Rabbit pAb\u003c\/p\u003e\n\n\u003cp\u003eSizes: 20μL, 100μL\u003c\/p\u003e\n\n\u003cp\u003eCatalogue Numbers: A21588-20, A21588-100\u003c\/p\u003e\n\n\u003cp\u003eCitations, Manuals and MSDS Available upon request.\u003c\/p\u003e\n\n\u003cp\u003eBackground: The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.\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: 50kDa\u003c\/p\u003e\n\n\u003cp\u003eObserved Molecular Weight: 50kDa\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: 593\u003c\/p\u003e\n\n\u003cp\u003eSWISS: P12694\u003c\/p\u003e\n\n\u003cp\u003eAlternate Names: MSU; MSUD1; OVD1A; BCKDE1A; BCKDHA\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 containing 50% glycerol, preserved with proclin300 or sodium azide (as specified on the Certificate of Analysis),  pH 7.3.\u003c\/p\u003e\n\n\u003cp\u003eRecommended Dilutions: WB, 1:500 - 1:1000 IF\/ICC, 1:50 - 1:200 ELISA, Recommended starting concentration is 1 μg\/mL. Please optimize the concentration based on your specific assay requirements.\u003c\/p\u003e\n\n\u003cp\u003eNCBI Alias: BCKDHA\u003c\/p\u003e\n\n\u003cp\u003eResearch Use Only\u003c\/p\u003e","brand":"ABclonal","offers":[{"title":"20uL","offer_id":45189403869363,"sku":"A21588-20","price":151.2,"currency_code":"CAD","in_stock":true},{"title":"100uL","offer_id":45189403902131,"sku":"A21588-100","price":417.2,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0305\/9482\/6376\/files\/download_84c565b3-c4ea-4e1e-b359-8672fa3aca8c.png?v=1766426403","url":"https:\/\/afsbio.com\/products\/bckdha-rabbit-pab-a21588","provider":"AFSBio Inc.","version":"1.0","type":"link"}