在线日韩日本国产亚洲丨少妇伦子伦情品无吗丨欧美性猛交xxxx免费看蜜桃丨精品人妻系列无码一区二区三区丨亚洲精品无码不卡在线播放

Your Good Partner in Biology Research

  1. Home
  2. 抗體
  3. 多克隆抗體
  4. TTC19 Antibody, HRP conjugated

TTC19 Antibody, HRP conjugated

  • 中文名稱：
    TTC19兔多克隆抗體, HRP偶聯(lián)
  • 貨號：
    CSB-PA724947LB01HU
  • 規(guī)格：
    ￥880
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)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：
    HRP
  • 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(chǎn)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(chǎn)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

    顯示更多

    收起更多

  • 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(shù)據(jù)庫鏈接：

    HGNC: 26006

    OMIM: 613814

    KEGG: hsa:54902

    STRING: 9606.ENSP00000261647

    UniGene: Hs.462316



主站蜘蛛池模板： 精品久久久久久无码中文字幕一区| 777亚洲精品乱码久久久久久| 黑人巨大精品欧美一区二区| 少妇熟女视频一区二区三区| 欧美性猛交xxx嘿人猛交| 亚洲 欧美精品suv| 久久久国产精品| 99久久无码私人网站| 18禁无遮挡无码网站免费| 久久99精品国产99久久6| 中文字幕资源网| 五月花成人网| 免费无码久久成人影片| 国产交换配乱婬视频偷网站| 中字幕一区二区三区乱码| 香蕉视频在线观看亚洲| 亚洲性色av一区二区三区| 日日摸日日碰夜夜爽无码| 免费人成视频| 国产精品一区二区久久| 99精品国产福利在线观看| 肉色丝袜足j视频国产| 成人区人妻精品一区二区三区| 欧美人与动牲交zooz乌克兰| 亚洲中文字幕日产乱码在线| 免费天堂无码人妻成人av电影| 成熟丰满熟妇高潮xxxxx| 久久不见久久见免费影院www日本| 久久国产精品2020免费| 2019日韩中文字幕mv| 国产成人精品日本亚洲77上位| 久久伊99综合婷婷久久伊| 欧美黑人xxxx性高清版| 美女视频黄是免费| 亚洲成av人在线视猫咪| 亚洲国产aⅴ综合网| 亚洲综合另类小说色区一| 又色又爽又高潮免费视频国产| 色六月婷婷亚洲婷婷六月| 久久人人爽人人爽人人片av高清| 久久久免费精品re6|