PDHX Antibody
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中文名稱：PDHX兔多克隆抗體
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貨號：CSB-PA017718GA01HU
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規格：￥3,900
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其他：
產品詳情
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Uniprot No.：
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基因名：PDHX
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別名：Dihydrolipoamide dehydrogenase binding protein of pyruvate dehydrogenase complex antibody; Dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex antibody; DLDBP antibody; E3 binding protein antibody; E3-binding protein antibody; E3BP antibody; Lipoyl containing pyruvate dehydrogenase complex component X antibody; Lipoyl-containing pyruvate dehydrogenase complex component X antibody; mitochondrial antibody; ODPX_HUMAN antibody; OPDX antibody; PDHX antibody; PDX 1 antibody; PDX1 antibody; Pro X antibody; proX antibody; Pyruvate dehydrogenase complex component X antibody; Pyruvate dehydrogenase complex lipoyl containing component X antibody; Pyruvate dehydrogenase complex; E3 binding protein subunit antibody; Pyruvate dehydrogenase protein X component antibody; Pyruvate dehydrogenase protein X component mitochondrial antibody
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宿主：Rabbit
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反應種屬：Human,Mouse,Rat
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免疫原：Human PDHX
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免疫原種屬：Homo sapiens (Human)
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抗體亞型：IgG
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純化方式：Antigen Affinity Purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產品提供形式：Liquid
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應用范圍：ELISA,WB,IHC
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Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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貨期：Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
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靶點詳情
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功能：Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex.
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基因功能參考文獻：
- We propose testing for the R446* mutation in PDHX as a rapid first screening in Roma infants with metabolic acidosis. PMID: 25087164
- MiR-26a regulates glucose metabolism of colorectal cancer cells by direct targeting PDHX. PMID: 24935220
- New mutation in PDHX gene found in two unrelated patients with Pyruvate dehydrogenase deficiency. PMID: 22766002
- genetic association with systemic lupus erythematosus to a haplotype between PDHX and CD44 was established. PMID: 21194677
- determination that PDH and complex III exist at a steady-state ratio of 1:100, 1:128 and 1:202 in HeLa cell extracts, fibroblast mitochondria and heart tissue mitochondria, respectively PMID: 12372595
- model of the pyruvate dehydrogenase complex formed by E2 and E2 plus the E3-binding protein and binding of the E1 and E3 components PMID: 14638692
- specificity of pairing for human E3BP with E3 from its subcomplex structure to be most likely due to conformational rigidity of the binding fragment of the E3-binding domain of E3BP and its exquisite amino acid match with the E3 target interface PMID: 16263718
- A cluster of disease-causing E3 mutations located near the center of the E3BD/E3 interface prevents the efficient recruitment of these E3 variants by E3BP into the PDC, leading to the dysfunction of the PDC catalytic machine. PMID: 16442803
- These data provide an additional case of E3BP deficiency with a unique and previously unreported deletion in the PDHX gene. PMID: 16566017
- Despite the presence of antibodies reactive with PDC-E3BP in the majority of primary biliary cirrhosis (PBC) patients this self-protein is not a dominant T-cell autoantigen in PBC. PMID: 16629643
- PDHX-assisted photosensitization with rose Bengal induces structural and functional alteration of mitochondria in HeLa cells. PMID: 17024456
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相關疾病：Pyruvate dehydrogenase E3-binding protein deficiency (PDHXD)
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亞細胞定位：Mitochondrion matrix.
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蛋白家族：2-oxoacid dehydrogenase family
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數據庫鏈接：
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