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TSFM Antibody

  • 中文名稱：
    TSFM兔多克隆抗體
  • 貨號：
    CSB-PA025124GA01HU
  • 規格：
    ￥3,900
  • 其他：

產品詳情

  • Uniprot No.：
  • 基因名：
    TSFM
  • 別名：
    COXPD 3 antibody; COXPD3 antibody; EF TS antibody; EF Tsmt antibody; EF-Ts antibody; EF-TsMt antibody; EFTS antibody; EFTS_HUMAN antibody; EFTSMT antibody; Elongation factor Ts antibody; Elongation factor Ts mitochondrial antibody; HGNC12367 antibody; mitochondrial antibody; Mitochondrial elongation factor Ts antibody; Ts translation elongation factor mitochondrial antibody; TSFM antibody; TSMT antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human,Mouse,Rat
  • 免疫原：
    Human TSFM
  • 免疫原種屬：
    Homo sapiens (Human)
  • 抗體亞型：
    IgG
  • 純化方式：
    Antigen Affinity Purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 產品提供形式：
    Liquid
  • 應用范圍：
    ELISA,WB,IHC
  • 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：
    Associates with the EF-Tu.GDP complex and induces the exchange of GDP to GTP. It remains bound to the aminoacyl-tRNA.EF-Tu.GTP complex up to the GTP hydrolysis stage on the ribosome.
  • 基因功能參考文獻：
    1. Different TSFM mutations can produce the same or very different clinical phenotypes, going from abortions to moderately severe presentations. On the other hand, the same TSFM mutation can also produce same or different phenotypes within the same range of presentations, therefore suggesting the involvement of unknown factors. PMID: 27677415
    2. show that in addition to early-onset cardiomyopathy, TSFM mutations should be considered in childhood and juvenile encephalopathies with optic and/or peripheral neuropathy, ataxia, or Leigh disease PMID: 25037205
    3. identified a homozygous mutation changing a highly conserved arginine into a tryptophan (R312W) in a kindred with intrauterine growth retardation, neonatal lactic acidosis, liver dysfunction and multiple respiratory chain deficiency in muscle PMID: 21741925
    4. Molecular modeling showed that the Arg333Trp substitution disrupts local subdomain structure and the dimerization interface. PMID: 17033963
  • 相關疾病：
    Combined oxidative phosphorylation deficiency 3 (COXPD3)
  • 亞細胞定位：
    Mitochondrion.
  • 蛋白家族：
    EF-Ts family
  • 組織特異性：
    Expressed in all tissues, with the highest levels of expression in skeletal muscle, liver and kidney.
  • 數據庫鏈接：

    HGNC: 12367

    OMIM: 604723

    KEGG: hsa:10102

    UniGene: Hs.632704



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