TRNT1 Antibody, FITC conjugated
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中文名稱：TRNT1兔多克隆抗體, FITC偶聯
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貨號：CSB-PA836282LC01HU
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規格：￥880
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) TRNT1 Polyclonal antibody
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Uniprot No.：
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基因名：TRNT1
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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
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宿主：Rabbit
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反應種屬：Human
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免疫原：Recombinant Human CCA tRNA nucleotidyltransferase 1, mitochondrial protein (128-293AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：FITC
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：>95%, Protein G purified
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濃度：It differs from different batches. Please contact us to confirm it.
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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
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
產品評價
相關產品
靶點詳情
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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).
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基因功能參考文獻：
- In vitro studies of disease-linked variants of human tRNA nucleotidyltransferase reveal decreased thermal stability and altered catalytic activity. PMID: 29454993
- 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
- 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
- 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
- two non-syndromic retinitis pigmentosa pedigrees with segregating mutations in TRNT1 PMID: 26494905
- 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
- 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
- The discriminator base represents an important substrate recognition element for tRNA nucleotidyltransferases. PMID: 25958396
- 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
- 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
- human gene transcript CGI-47 (#AF151805) was cloned and encodes a bona fide CCA-adding enzyme and not a poly(A) polymerase. PMID: 11727826
- 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
- 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
- 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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相關疾病：Sideroblastic anemia with B-cell immunodeficiency, periodic fevers, and developmental delay (SIFD); Retinitis pigmentosa and erythrocytic microcytosis (RPEM)
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亞細胞定位：Mitochondrion.
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蛋白家族：TRNA nucleotidyltransferase/poly(A) polymerase family
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數據庫鏈接：
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