PDC-E2 Monoclonal Antibody

活动价:

¥880.00

市场价:¥1280.00

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产品规格:
  • 50μL
货号:

FAB2600954

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商品描述
  • 靶点-Target

    PDC-E2

  • 反应性

    Human,Rabbit

  • 应用

    WB

  • 宿主物种-Host Species

    Mouse

  • 修饰-Modification

    Unmodified

  • MW(Calculated)

    69kD

  • 推荐稀释比

    WB 1:1000-1:2000;Not yet tested in other applications.

  • 组成

    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

  • 纯化工艺

    Affinity purification

  • 储存

    -15°C to -25°C/1 year(Do not lower than -25°C)

  • 克隆性

    Monoclonal

  • 免疫原

    Purified recombinant human PDC-E2 protein fragments expressed in E.coli.

  • 特异性

    PDC-E2 Monoclonal Antibody detects endogenous levels of PDC-E2 protein.

  • 基因名称

    DLAT

  • 蛋白名称

    Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial

  • 别名

    DLAT;DLTA;Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex;mitochondrial;70 kDa mitochondrial autoantigen of primary biliary cirrhosis;PBC;Dihydrolipoamide acetyltransferase component of pyruva

  • Organism-1

    Human

  • 基因ID-1

    1737

  • SwissProt-1

    P10515

  • Organism-2

    Mouse

  • SwissProt-2

    Q8BMF4

  • Organism-3

    Rat

  • SwissProt-3

    P08461

  • 背景

    This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lact

  • 细胞定位

    Mitochondrion matrix.