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

TRNT1 Antibody

  • 中文名稱:
    TRNT1兔多克隆抗體
  • 貨號:
    CSB-PA836282LA01HU
  • 規格:
    ¥440
  • 圖片:
    • Western Blot
      Positive WB detected in: 293T whole cell lysate, HepG2 whole cell lysate
      All lanes: TRNT1 antibody at 5μg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 51, 48, 7 kDa
      Observed band size: 51, 48 kDa
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) TRNT1 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    TRNT1
  • 別名:
    TRNT1 antibody; CGI-47 antibody; CCA tRNA nucleotidyltransferase 1 antibody; mitochondrial antibody; EC 2.7.7.72 antibody; Mitochondrial tRNA nucleotidyl transferase antibody; CCA-adding antibody; mt CCA-adding enzyme antibody; mt tRNA CCA-diphosphorylase antibody; mt tRNA CCA-pyrophosphorylase antibody; mt tRNA adenylyltransferase antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human CCA tRNA nucleotidyltransferase 1, mitochondrial protein (128-293AA)
  • 免疫原種屬:
    Homo sapiens (Human)
  • 標記方式:
    Non-conjugated

    本頁面中的產品,TRNT1 Antibody (CSB-PA836282LA01HU),的標記方式是Non-conjugated。對于TRNT1 Antibody,我們還提供其他標記。見下表:

    可提供標記
    標記方式 貨號 產品名稱 應用
    HRP CSB-PA836282LB01HU TRNT1 Antibody, HRP conjugated ELISA
    FITC CSB-PA836282LC01HU TRNT1 Antibody, FITC conjugated
    Biotin CSB-PA836282LD01HU TRNT1 Antibody, Biotin conjugated ELISA
  • 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, WB
  • 推薦稀釋比:
    Application Recommended Dilution
    WB 1:500-1:5000
  • 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:
    Adds and repairs the conserved 3'-CCA sequence necessary for the attachment of amino acids to the 3' terminus of tRNA molecules, using CTP and ATP as substrates.; Adds 2 C residues (CC-) to the 3' terminus of tRNA molecules instead of a complete CCA end as isoform 1 does (in vitro).
  • 基因功能參考文獻:
    1. In vitro studies of disease-linked variants of human tRNA nucleotidyltransferase reveal decreased thermal stability and altered catalytic activity. PMID: 29454993
    2. patient-specific induced pluripotent stem cells (iPSCs) and iPSC-derived retinal organoids from dermal fibroblasts of patients with molecularly confirmed TRNT1-associated retinitis pigmentosa. PMID: 28390992
    3. Data show that the disease causing mutations in patient-derived fibroblasts do not affect subcellular localization of TRNT1 and show no gross morphological differences when compared to control cells. PMID: 27317422
    4. family expands the ocular and systemic phenotypes associated with mutations in TRNT1, demonstrating phenotypic variability and highlighting the need for ophthalmic review of these patients. PMID: 27389523
    5. two non-syndromic retinitis pigmentosa pedigrees with segregating mutations in TRNT1 PMID: 26494905
    6. The clinical phenotypes associated with TRNT1 mutations are largely due to impaired mitochondrial translation, resulting from defective CCA addition to mitochondrial tRNA(Ser(AGY)). PMID: 25652405
    7. A model of action is proposed, where motif C forms a flexible spring element modulating the relative orientation of the enzyme's head and body domains to accommodate the growing 3'-end of the tRNA. PMID: 25849199
    8. The discriminator base represents an important substrate recognition element for tRNA nucleotidyltransferases. PMID: 25958396
    9. Tandem CCA addition is not the result of a modified enzymatic activity that is particular to unstable RNAs. Rather, it is a consequence of the natural activity of the CCA-adding enzyme on a substrate with increased conformational flexibility, the CCA-adding enzyme is able to trigger the degradation of potentially detrimental small RNAs and tRNAs. PMID: 25640237
    10. The patient-associated TRNT1 mutations result in partial loss of function of TRNT1 and lead to metabolic defects in both the mitochondria and cytosol, which can account for the phenotypic pleiotropy. PMID: 25193871
    11. human gene transcript CGI-47 (#AF151805) was cloned and encodes a bona fide CCA-adding enzyme and not a poly(A) polymerase. PMID: 11727826
    12. The crystal structure reveals a four domain architecture with a cluster of conserved residues forming a positively charged cleft between the first two domains. PMID: 12729736
    13. These findings strongly suggest that the splice variant of the human CCA-adding enzyme is expressed in the cell although the in vivo function remains unclear. PMID: 17204286
    14. is a RNA polymerase which newly adds CCA sequence to tRNA 3'terminal. This reaction was named as Vice-Anchored Knock-in and Lock Dynamics.[review] PMID: 18575231

    顯示更多

    收起更多

  • 相關疾病:
    Sideroblastic anemia with B-cell immunodeficiency, periodic fevers, and developmental delay (SIFD); Retinitis pigmentosa and erythrocytic microcytosis (RPEM)
  • 亞細胞定位:
    Mitochondrion.
  • 蛋白家族:
    TRNA nucleotidyltransferase/poly(A) polymerase family
  • 數據庫鏈接:

    HGNC: 17341

    OMIM: 612907

    KEGG: hsa:51095

    STRING: 9606.ENSP00000251607

    UniGene: Hs.732725



主站蜘蛛池模板: 亚洲综合天堂一区二区三区 | 在线观看人成视频免费| 小说区 图片区色 综合区| 国产人澡人澡澡澡人碰视频| 亚洲高清无码加勒比| 高中国产开嫩苞实拍视频在线观看| 免费人成视频19674不收费| 超清中文乱码字幕在线观看| 婷婷丁香六月激情综合在线人| 亚洲一区二区无码影院| 中文字幕久热精品视频在线| 婷婷丁香社区| 久久99精品国产99久久6尤物| 免费无码又爽又刺激高潮的动态图| 亚洲 欧美 国产 动漫 综合| 东京热加勒比无码少妇| 国产精品久久久久久久久动漫| 99久久久无码国产精品动漫| 国产av丝袜一区二区三区| 亚洲国产精久久久久久久| 中文字幕乱码免费看电影| 美女把尿囗扒开让男人添| 欧美自拍亚洲综合丝袜| 国产免费牲交视频| 国产偷人妻精品一区| 蜜臀久久99精品久久久久久做爰| 欧美日韩亚洲国产在线制服| 风韵少妇性饥渴推油按摩视频| 国产亚洲精品97在线视频一| 国产午夜精华2020在线| 久久久久久无码av成人影院| 国产无遮挡裸体免费视频在线观看 | 亚洲bbw性色大片| 亚洲第一女人av| 亚洲一本大道av久在线播放| 亚洲 一区二区 在线| 狠狠躁18三区二区一区| 日本高清www午色夜com| 9999国产精品欧美久久久久久| 人妻熟女一区二区三区app下载| 日产一区日产2区|