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TRNT1 Antibody, FITC conjugated

  • 中文名稱：
    TRNT1兔多克隆抗體, FITC偶聯(lián)
  • 貨號：
    CSB-PA836282LC01HU
  • 規(guī)格：
    ￥880
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)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：
    FITC
  • 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
  • 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.

產(chǎn)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

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  • 相關疾?。?/div>
    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(shù)據(jù)庫鏈接：

    HGNC: 17341

    OMIM: 612907

    KEGG: hsa:51095

    STRING: 9606.ENSP00000251607

    UniGene: Hs.732725