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SDHAF1 Antibody, FITC conjugated

  • 中文名稱：
    SDHAF1兔多克隆抗體, FITC偶聯
  • 貨號：
    CSB-PA406800LC01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) SDHAF1 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
  • 別名：
    LYR motif containing 8 antibody; LYR motif-containing protein 8 antibody; LYRM8 antibody; mitochondrial antibody; SDH assembly factor 1 antibody; SDHAF1 antibody; SDHF1_HUMAN antibody; Succinate dehydrogenase assembly factor 1 antibody; Succinate dehydrogenase assembly factor 1 mitochondrial antibody; Succinate dehydrogenase complex assembly factor 1 antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Succinate dehydrogenase assembly factor 1, mitochondrial protein (78-115AA)
  • 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：
    Plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol. Promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants. May act together with SDHAF3. Contributes to iron-sulfur cluster incorporation into SDHB by binding to SDHB and recruiting the iron-sulfur transfer complex formed by HSC20, HSPA9 and ISCU through direct binding to HSC20.
  • 基因功能參考文獻：
    1. that SDHAF1 contributes to iron-sulfur (Fe-S) cluster incorporation into the Fe-S subunit of CII, SDHB. PMID: 26749241
    2. Studies indicate that an array of tumor syndromes caused by complex II-associated mutations in genes SDHA, SDHB, SDHC, SDHD, SDHAF1 and SDHAF2 have been identified over a decade. PMID: 23174333
    3. Leukoencephalopathy with accumulated succinate is a key symptom of defective complex II assembly due to SDHAF1 mutations PMID: 22995659
  • 相關疾病：
    Mitochondrial complex II deficiency (MT-C2D)
  • 亞細胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Complex I LYR family, SDHAF1 subfamily
  • 組織特異性：
    Ubiquitously expressed.
  • 數據庫鏈接：

    HGNC: 33867

    OMIM: 252011

    KEGG: hsa:644096

    STRING: 9606.ENSP00000368165

    UniGene: Hs.356460