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ACAD8 Antibody

  • 中文名稱:
    ACAD8兔多克隆抗體
  • 貨號:
    CSB-PA798115
  • 規(guī)格:
    ¥1100
  • 圖片:
    • The image on the left is immunohistochemistry of paraffin-embedded Human colon cancer tissue using CSB-PA798115(ACAD8 Antibody) at dilution 1/15, on the right is treated with fusion protein. (Original magnification: ×200)
    • The image on the left is immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using CSB-PA798115(ACAD8 Antibody) at dilution 1/15, on the right is treated with fusion protein. (Original magnification: ×200)
  • 其他:

產(chǎn)品詳情

  • Uniprot No.:
  • 基因名:
    ACAD8
  • 別名:
    ACAD 8 antibody; ACAD-8 antibody; ACAD8 antibody; ACAD8_HUMAN antibody; Activator-recruited cofactor 42 kDa component antibody; Acyl CoA dehydrogenase family member 8 antibody; Acyl Coenzyme A dehydrogenase family member 8 antibody; Acyl-CoA dehydrogenase family member 8 antibody; ARC42 antibody; FLJ22590 antibody; IBD antibody; Isobutyryl-CoA dehydrogenase antibody; Isobutyryl-CoA dehydrogenase; mitochondrial antibody; mitochondrial antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human,Mouse
  • 免疫原:
    Fusion protein of Human ACAD8
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated
  • 抗體亞型:
    IgG
  • 純化方式:
    Antigen affinity purification
  • 濃度:
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液:
    -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
  • 產(chǎn)品提供形式:
    Liquid
  • 應用范圍:
    ELISA,IHC
  • 推薦稀釋比:
    Application Recommended Dilution
    ELISA 1:1000-1:2000
    IHC 1:15-1:50
  • 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.

產(chǎn)品評價

靶點詳情

  • 功能:
    Isobutyryl-CoA dehydrogenase which catalyzes one of the steps of the valine catabolic pathway. To a lesser extent, is also able to catalyze the oxidation of (2S)-2-methylbutanoyl-CoA.
  • 基因功能參考文獻:
    1. we discovered a novel c.1156_1158delCAG mutation in ACAD8 in patients with isobutyryl-CoA dehydrogenase deficiency , and investigated the mutation spectrum of ACAD8. PMID: 24635911
    2. first enzymatic and molecular confirmation of a deficiency of this enzyme in a patient PMID: 12359132
  • 相關(guān)疾病:
    Isobutyryl-CoA dehydrogenase deficiency (IBDD)
  • 亞細胞定位:
    Mitochondrion.
  • 蛋白家族:
    Acyl-CoA dehydrogenase family
  • 組織特異性:
    Detected at comparable levels in all tissues examined (heart, lung, brain, skeletal muscle, pancreas and placenta). Weakly expressed in liver and kidney.
  • 數(shù)據(jù)庫鏈接:

    HGNC: 87

    OMIM: 604773

    KEGG: hsa:27034

    STRING: 9606.ENSP00000281182

    UniGene: Hs.14791