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  4. IBA57 Antibody, Biotin conjugated

IBA57 Antibody, Biotin conjugated

  • 中文名稱：
    IBA57兔多克隆抗體, Biotin偶聯
  • 貨號：
    CSB-PA722566LD01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) IBA57 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    IBA57
  • 別名：
    C1orf69 antibody; CAF17_HUMAN antibody; Chromosome 1 open reading frame 69 antibody; FLJ12734 antibody; FLJ13849 antibody; IBA57 antibody; IBA57 homolog iron sulfur cluster assembly antibody; IBA57 iron sulfur cluster assembly homolog antibody; IBA57 iron sulfur cluster assembly homolog (S. cerevisiae) antibody; IBA57; S. cerevisiae; homolog of antibody; Iron sulfur cluster assembly factor for biotin synthase and aconitase like antibody; iron-sulfur cluster assembly factor for biotin synthase- and aconitase-like mitochondrial proteins; with a mass of 57kDa antibody; Iron-sulfur cluster assembly factor homolog antibody; Mitochondrial proteins with a mass of 57kDa antibody; MMDS3 antibody; Putative transferase C1orf69 mitochondrial antibody; Putative transferase CAF17; mitochondrial antibody; SPG74 antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Putative transferase CAF17, mitochondrial protein (286-356AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Biotin
  • 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
  • 應用范圍：
    ELISA
  • 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.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 功能：
    Involved in the maturation of mitochondrial 4Fe-4S proteins functioning late in the iron-sulfur cluster assembly pathway.
  • 基因功能參考文獻：
    1. a patient with infantile-onset optic atrophy and asymptomatic white matter involvement, thus broadening the phenotypic spectrum of biallelic IBA57 mutations. PMID: 30258207
    2. Description of four unrelated patients carrying novel mutations in IBA57; study expands the array of the genotypic variation of IBA57 and delineates the leukodystrophic pattern of IBA57 deficient patients PMID: 27785568
    3. Findings reinforce the suggested specific function of IBA57 in mitochondrial [4Fe-4S] protein maturation and provide additional evidence for its role in hereditary spastic paraplegia PMID: 25609768
    4. Mutation of the iron-sulfur cluster assembly gene IBA57 causes severe myopathy and encephalopathy. PMID: 23462291
  • 相關疾病：
    Multiple mitochondrial dysfunctions syndrome 3 (MMDS3); Spastic paraplegia 74, autosomal recessive (SPG74)
  • 亞細胞定位：
    Mitochondrion.
  • 蛋白家族：
    GcvT family, CAF17 subfamily
  • 組織特異性：
    Expressed in skin fibroblasts and skeletal muscle (at protein level).
  • 數據庫鏈接：

    HGNC: 27302

    OMIM: 615316

    KEGG: hsa:200205

    STRING: 9606.ENSP00000355672

    UniGene: Hs.237017



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