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PDHX Antibody

  • 中文名稱:
    PDHX兔多克隆抗體
  • 貨號:
    CSB-PA017718GA01HU
  • 規格:
    ¥3,900
  • 其他:

產品詳情

  • Uniprot No.:
  • 基因名:
    PDHX
  • 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
  • 宿主:
    Rabbit
  • 反應種屬:
    Human,Mouse,Rat
  • 免疫原:
    Human PDHX
  • 免疫原種屬:
    Homo sapiens (Human)
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen Affinity Purified
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產品提供形式:
    Liquid
  • 應用范圍:
    ELISA,WB,IHC
  • Protocols:
  • 儲存條件:
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 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.
  • 用途:
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 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.
  • 基因功能參考文獻:
    1. We propose testing for the R446* mutation in PDHX as a rapid first screening in Roma infants with metabolic acidosis. PMID: 25087164
    2. MiR-26a regulates glucose metabolism of colorectal cancer cells by direct targeting PDHX. PMID: 24935220
    3. New mutation in PDHX gene found in two unrelated patients with Pyruvate dehydrogenase deficiency. PMID: 22766002
    4. genetic association with systemic lupus erythematosus to a haplotype between PDHX and CD44 was established. PMID: 21194677
    5. 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
    6. 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
    7. 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
    8. 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
    9. These data provide an additional case of E3BP deficiency with a unique and previously unreported deletion in the PDHX gene. PMID: 16566017
    10. 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
    11. 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)
  • 亞細胞定位:
    Mitochondrion matrix.
  • 蛋白家族:
    2-oxoacid dehydrogenase family
  • 數據庫鏈接:

    HGNC: 21350

    OMIM: 245349

    KEGG: hsa:8050

    STRING: 9606.ENSP00000227868

    UniGene: Hs.502315



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