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PDHX Antibody

  • 货号：
    CSB-PA017718ESR2HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western blot
      All lanes: PDHX antibody at 6μg/ml
      Lane 1: 293T whole cell lysate
      Lane 2: PC-3 whole cell lysate
      Lane 3: MCF-7 whole cell lysate
      Secondary
      Goat polyclonal to rabbit IgG at 1/10000 dilution
      Predicted band size: 55, 30, 52 kDa
      Observed band size: 55 kDa
    • Immunohistochemistry of paraffin-embedded human tonsil tissue using CSB-PA017718ESR2HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human placenta tissue using CSB-PA017718ESR2HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human colon tissue using CSB-PA017718ESR2HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) PDHX Polyclonal antibody
  • Uniprot No.：
    O00330
  • 基因名：
    PDHX
  • 别名：
    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
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Pyruvate dehydrogenase protein X component, mitochondrial protein (1-300AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:20-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Pyruvate dehydrogenase E3-binding protein deficiency (PDHXD)
  • 亚细胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    2-oxoacid dehydrogenase family
  • 数据库链接：

    HGNC: 21350

    OMIM: 245349

    KEGG: hsa:8050

    STRING: 9606.ENSP00000227868

    UniGene: Hs.502315