TRNT1 Antibody, Biotin conjugated

說明書

中文名稱：

TRNT1兔多克隆抗體, Biotin偶聯
貨號：

CSB-PA836282LD01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) TRNT1 Polyclonal antibody
Uniprot No.：

Q96Q11
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：

Biotin
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
Protocols：

ELISA Protocol
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
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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)
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

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