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  4. ACAD8 Antibody, FITC conjugated

ACAD8 Antibody, FITC conjugated

  • 中文名稱：
    ACAD8兔多克隆抗體, FITC偶聯
  • 貨號：
    CSB-PA892457LC01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) ACAD8 Polyclonal antibody
  • 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
  • 免疫原：
    Recombinant Human Isobutyryl-CoA dehydrogenase, mitochondrial protein (201-415AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    FITC
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 產品提供形式：
    Liquid
  • 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：
    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
  • 相關疾?。?/div>
    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.
  • 數據庫鏈接：

    HGNC: 87

    OMIM: 604773

    KEGG: hsa:27034

    STRING: 9606.ENSP00000281182

    UniGene: Hs.14791



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