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  4. TTC19 Antibody, Biotin conjugated

TTC19 Antibody, Biotin conjugated

  • 中文名稱：
    TTC19兔多克隆抗體, Biotin偶聯
  • 貨號：
    CSB-PA724947LD01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) TTC19 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    TTC19
  • 別名：
    2010204O13Rik antibody; FLJ20343 antibody; MGC138312 antibody; MGC19520 antibody; mitochondrial antibody; RIKEN cDNA 2810460C24 antibody; Tetratricopeptide repeat protein 19 antibody; TPR repeat protein 19 antibody; Ttc 19 antibody; TTC19 antibody; TTC19_HUMAN antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Tetratricopeptide repeat protein 19, mitochondrial protein (108-380AA)
  • 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：
    Required for the preservation of the structural and functional integrity of mitochondrial respiratory complex III by allowing the physiological turnover of the Rieske protein UQCRFS1. Involved in the clearance of UQCRFS1 N-terminal fragments, which are produced upon incorporation of UQCRFS1 into the complex III and whose presence is detrimental for its catalytic activity.
  • 基因功能參考文獻：
    1. TTC19 preserves the structural and functional integrity of mitochondrial respiratory complex III. UQCRFS1 produces N-terminal polypeptides, which remain bound to holocomplex III. UQCRFS1 fragments are rapidly removed, but when TTC19 is absent they accumulate within complex III, causing its structural and functional impairment. PMID: 28673544
    2. TTC19-deficient mitochondrial complex III deficiency displays substantial phenotypic variation. (Review) PMID: 25899669
    3. This study showed that TTC19 deficient patients do show characteristic clinical and neuroimaging features, which may facilitate diagnosis of this yet rare disorder; normal MRC complex III activity does not exclude the diagnosis. PMID: 25887401
    4. A TTC19 mutation in spinocerebellar ataxia is identified in an Asian population. PMID: 24397319
    5. The mutation resulted in almost complete absence of TTC19 protein, defective assembly of CIII in muscle, and enhanced production of reactive oxygen species in cultured skin fibroblasts. PMID: 23532514
    6. TTC19 is a putative cIII assembly factor whose disruption is associated with severe neurological abnormalities in humans and flies. PMID: 21278747

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  • 相關疾病：
    Mitochondrial complex III deficiency, nuclear 2 (MC3DN2)
  • 亞細胞定位：
    Mitochondrion inner membrane.
  • 蛋白家族：
    TTC19 family
  • 數據庫鏈接：

    HGNC: 26006

    OMIM: 613814

    KEGG: hsa:54902

    STRING: 9606.ENSP00000261647

    UniGene: Hs.462316



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