{"product_id":"proteintech-13685-1-ap","title":"Proteintech, 13685-1-AP, BCKDHB Polyclonal antibody","description":"Size: 20ul \/ 150ul\nThe BCKDHB (13685-1-AP) by Proteintech is a Polyclonal antibody targeting BCKDHB in WB, IHC, ELISA applications with reactivity to human, mouse, rat samples\n13685-1-AP targets BCKDHB in WB, IHC, RIP, ELISA applications and shows reactivity with human, mouse, rat samples.\n\u003cb\u003eTested Applications\u003c\/b\u003e\nPositive WB detected in: mouse liver tissue, Transfected HEK-293 cells,  rat liver tissue\nPositive IHC detected in: human colon cancer tissue, human stomach tissueNote: suggested antigen retrieval withTE buffer pH 9.0;(*) Alternatively, antigen retrieval may be performed withcitrate buffer pH 6.0\n\u003cb\u003eRecommended dilution\u003c\/b\u003e\nWestern Blot (WB): WB : 1:500-1:2000\nImmunohistochemistry (IHC): IHC : 1:50-1:500\n\u003cb\u003eBackground Information\u003c\/b\u003e\nBranched-chain alpha-keto acid dehydrogenase E1 component beta chain (BCKDHB; BCKDE1B; BCKDH E1-beta) is a subunit of the BCKDH complex, which is a mitochondrial enzyme in the degradation pathway for branched-chain amino acids (BCAA). Together with BCKDHA, BCKDHB forms the E1 subunit of this complex, whereas DBT and DLD are the E2 and E3 subunits, respectively. A deficiency of the BCKDH complex in humans causes maple syrup urine disease (MSUD), a severe neurometabolic disorder diagnosed by the detection alloisoleucine in plasma (MIM 248600). (PMID: 30709776)\n\u003cb\u003eSpecification\u003c\/b\u003e\nTested Reactivity: human, mouse, rat\nCited Reactivity: human, mouse, sheep\nHost \/ Isotype: Rabbit \/ IgG\nClass: Polyclonal\nType: Antibody\nImmunogen: CatNo: Ag4552 Product name: Recombinant human BCKDHB protein Source: e coli. -derived, PGEX-4T Tag: GST Domain: 41-392 aa of BC040139 Sequence: TVEDAAQRRQVAHFTFQPDPEPREYGQTQKMNLFQSVTSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVFNTPLCEQGIVGFGIGIAVTGATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSLTIRSPWGCVGHGALYHSQSPEAFFAHCPGIKVVIPRSPFQAKGLLLSCIEDKNPCIFFEPKILYRAAAEEVPIEPYNIPLSQAEVIQEGSDVTLVAWGTQVHVIREVASMAKEKLGVSCEVIDLRTIIPWDVDTICKSVIKTGRLLISHEAPLTGGFASEISSTVQEECFLNLEAPISRVCGYDTPFPHIFEPFYIPDKWKCYDALRKMINY Predict reactive species\nFull Name: branched chain keto acid dehydrogenase E1, beta polypeptide\nCalculated Molecular Weight: 392 aa, 43 kDa\nObserved Molecular Weight: 37 kDa\nGenBank Accession Number: BC040139\nGene Symbol: BCKDHB\nGene ID (NCBI): 594\nRRID: AB_2877972\nConjugate: Unconjugated\nForm: Liquid\nPurification Method: Antigen affinity purification\nUNIPROT ID: P21953\nStorage Buffer: PBS with 0.02% sodium azide and 50% glycerol, pH 7.3.\nStorage Conditions: Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20 o C storage. 20ul sizes contain 0.1% BSA.","brand":"Proteintech","offers":[{"title":"Default Title","offer_id":46868923089065,"sku":"13685-1-AP","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/proteintech-13685-1-ap","provider":"Iright","version":"1.0","type":"link"}