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TTC19 Antibody

  • 货号：
    CSB-PA724947LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human cervical cancer using CSB-PA724947LA01HU at dilution of 1:100
    • Immunofluorescent analysis of MCF-7 cells using CSB-PA724947LA01HU at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L)
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) TTC19 Polyclonal antibody
  • Uniprot No.：
    Q6DKK2
  • 基因名：
    TTC19
  • 别名：
    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
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Tetratricopeptide repeat protein 19, mitochondrial protein (108-380AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，TTC19 Antibody (CSB-PA724947LA01HU)，的标记方式是Non-conjugated。对于TTC19 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA724947LB01HU TTC19 Antibody, HRP conjugated ELISA
    FITC CSB-PA724947LC01HU TTC19 Antibody, FITC conjugated
    Biotin CSB-PA724947LD01HU TTC19 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:20-1:200
    IF 1:50-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Mitochondrial complex III deficiency, nuclear 2 (MC3DN2)
  • 亚细胞定位：
    Mitochondrion inner membrane.
  • 蛋白家族：
    TTC19 family
  • 数据库链接：

    HGNC: 26006

    OMIM: 613814

    KEGG: hsa:54902

    STRING: 9606.ENSP00000261647

    UniGene: Hs.462316