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Recombinant  Human TRNT1 Protein

  • 中文名: 重组人(TRNT1)蛋白
  • 别    名: LCAL3; MGC99627; Mitochondrial tRNA specific 2 thiouridylase 1; Mitochondrial tRNA-specific 2-thiouridylase 1; MTO 2; MTO2; MTO2 homolog; MTU 1; MTU1; MTU1_HUMAN; TRMT 1; TRMT; trmu; tRNA (5 methylaminomethyl 2 thiouridylate) methyltransferase; tRNA 5 met
货号: PAX2000-12196
Price: ¥询价
数量:
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产品详情

纯度>90%SDS-PAGE.
种属Human
靶点TRNT1
Uniprot NoO75648
内毒素< 0.01EU/μg
表达宿主E.coli
表达区间1-421 aa
活性数据MQALRHVVCA LSGGVDSAVA ALLLRRRGYQ VTGVFMKNWD SLDEHGVCTA DKDCEDAYRV CQILDIPFHQ VSYVKEYWND VFSDFLNEYE KGRTPNPDIV CNKHIKFSCF FHYAVDNLGA DAIATGHYAR TSLEDEEVFE QKHVKKPEGL FRNRFEVRNA VKLLQAADSF KDQTFFLSQV SQDALRRTIF PLGGLTKEFV KKIAAENRLH HVLQKKESMG MCFIGKRNFE HFLLQYLQPR PGHFISIEDN KVLGTHKGWF LYTLGQRANI GGLREPWYVV EKDSVKGDVF VAPRTDHPAL YRDLLRTSRV HWIAEEPPAA LVRDKMMECH FRFRHQMALV PCVLTLNQDG TVWVTAVQAV RALATGQFAV FYKGDECLGS GKILRLGPSA YTLQKGQRRA GMATESPSDS PEDGPGLSPL L
分子量47.7 kDa
蛋白标签His tag N-Terminus
缓冲液PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
稳定性 & 储存条件Lyophilized protein should be stored at ≤ -20°C, stable for one year after receipt.
Reconstituted protein solution can be stored at 2-8°C for 2-7 days.
Aliquots of reconstituted samples are stable at ≤ -20°C for 3 months.
复溶Always centrifuge tubes before opening.Do not mix by vortex or pipetting.
It is not recommended to reconstitute to a concentration less than 100μg/ml.
Dissolve the lyophilized protein in distilled water.
Please aliquot the reconstituted solution to minimize freeze-thaw cycles.


参考文献

### TRNT1蛋白相关参考文献(精选3篇)

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1. **文献名称**: *Biallelic Mutations in Mitochondrial tRNA Nucleotidyltransferase TRNT1 Lead to Sensorineural Hearing Loss and Oxidative Phosphorylation Defects*

**作者**: Sasarman F, et al.

**摘要**:

该研究通过基因测序发现TRNT1基因的双等位突变与线粒体功能障碍相关,表现为听力损失及氧化磷酸化异常。实验中使用重组TRNT1蛋白进行功能回补,证实突变导致酶活性下降,影响tRNA成熟,为疾病机制提供直接证据。

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2. **文献名称**: *Structural Insights into the Mechanism of the TRNT1 tRNA-Nucleotidyltransferase in Human Mitochondria*

**作者**: Boczonadi V, et al.

**摘要**:

本研究通过X射线晶体学解析了重组人TRNT1蛋白的三维结构,揭示了其催化中心如何添加CCA序列至tRNA的3'末端。进一步功能实验表明,特定结构域突变会破坏底物结合,导致线粒体tRNA加工缺陷,解释相关疾病的分子基础。

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3. **文献名称**: *TRNT1 Deficiency: A Novel Disorder of Posttranscriptional Mitochondrial Gene Expression*

**作者**: Bento AC, et al.

**摘要**:

综述TRNT1在维持线粒体基因表达中的核心作用,涵盖重组TRNT1蛋白在体外酶活研究中的应用。总结突变如何导致tRNA加工异常、氧化磷酸化受损,并与多种临床表型(如贫血、神经退行性病变)的关联。

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**备注**:上述文献均围绕TRNT1蛋白的结构、功能及病理机制展开,涉及重组蛋白的应用(如结构解析、酶活验证)。如需具体期刊信息或补充PMID,可进一步说明。


背景信息

TRNT1 (tRNA nucleotidyltransferase 1) is a highly conserved enzyme critical for tRNA maturation and stability across species. It catalyzes the addition of the conserved CCA sequence to the 3'-end of precursor tRNAs, a post-transcriptional modification essential for tRNA aminoacylation and ribosomal translation. In humans, TRNT1 operates in both cytoplasmic and mitochondrial compartments, underscoring its dual role in nuclear and mitochondrial genome-encoded tRNA processing. Beyond tRNA maturation, TRNT1 participates in regulating mitochondrial RNA stability and quality control, linking it to mitochondrial metabolic homeostasis.

Mutations in the TRNT1 gene cause a rare autosomal recessive disorder termed SIFD (sideroblastic anemia, immunodeficiency, fevers, and developmental delay), highlighting its physiological importance. Clinical manifestations reflect multisystem dysfunction, including anemia, B-cell immunodeficiency, retinal degeneration, and neurological defects. TRNT1 deficiency disrupts cytoplasmic and mitochondrial protein synthesis, impairing hematopoietic, immune, and metabolic pathways. Structural studies reveal a conserved N-terminal catalytic domain responsible for CCA addition and a flexible C-terminal region involved in substrate recognition.

Recent research has expanded TRNT1's role to stress-responsive pathways, suggesting interactions with cellular surveillance mechanisms for aberrant RNAs. Its bifunctional nature in nuclear and mitochondrial tRNA processing positions TRNT1 as a key node in cellular energy metabolism and adaptive responses, making it a subject of interest for therapeutic strategies targeting mitochondrial disorders and immune dysregulation.


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