| WB | 1/1000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | MCOPS5; MGC45000 |
| Entrez GeneID | 5015 |
| clone | 1H12C4B5 |
| WB Predicted band size | 32kDa |
| Host/Isotype | Mouse IgG1 |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human,Mouse,Rat,Rabbit,Monkey |
| Immunogen | Purified recombinant fragment of human OTX2 expressed in E. Coli. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于GTPBP3抗体的3篇假设性参考文献(请注意，以下为示例，实际文献需通过学术数据库验证)：
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1. **文献名称**: *"GTPBP3 mutations disrupt mitochondrial tRNA modification and cause combined oxidative phosphorylation deficiency"*
**作者**: Armengod, M.E., et al. (2014)
**摘要**: 本研究揭示了GTPBP3在线粒体tRNA硫修饰中的关键作用，其基因突变通过抗体检测证实与线粒体功能障碍相关，导致氧化磷酸化系统异常及儿童神经退行性疾病。
2. **文献名称**: *"Expression and functional analysis of GTPBP3 in mammalian cellular models using specific antibodies"*
**作者**: Zhou, X., et al. (2016)
**摘要**: 通过制备GTPBP3特异性抗体，作者验证了其在HeLa细胞和小鼠组织中的表达与定位，发现GTPBP3缺失导致线粒体tRNA结构异常，影响翻译效率。
3. **文献名称**: *"Antibody-based detection of GTPBP3 deficiency in mitochondrial disease diagnostics"*
**作者**: Khan, N.A., et al. (2020)
**摘要**: 开发了一种基于GTPBP3抗体的ELISA检测方法，用于快速筛查患者样本中的GTPBP3蛋白水平异常，为线粒体疾病的分子诊断提供新工具。
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**注意**：以上文献为示例，实际引用需查询PubMed、Web of Science等平台。建议使用关键词“GTPBP3 antibody”或“GTPBP3 mitochondrial function”检索近期研究。
GTPBP3 (GTP-binding protein 3) is a mitochondrial protein involved in post-transcriptional modification of transfer RNAs (tRNAs), specifically facilitating the 5-taurinomethyl modification of mitochondrial tRNA^(Gln), tRNA^(Glu), and tRNA^(Lys). This modification is critical for proper tRNA stability, structural integrity, and accurate mitochondrial translation. Defects in GTPBP3 are linked to mitochondrial disorders, such as combined oxidative phosphorylation deficiency 1 (COXPD1), characterized by impaired energy production, hypertrophic cardiomyopathy, and lactic acidosis.
The GTPBP3 (Center) antibody is a targeted immunological tool designed to detect the central region of the human GTPBP3 protein. It is commonly used in research to study mitochondrial tRNA maturation, respiratory chain dysfunction, and disease mechanisms. Validated applications include Western blotting, immunofluorescence, and immunohistochemistry, enabling localization of GTPBP3 within mitochondrial compartments. Researchers also employ this antibody to investigate tissue-specific expression patterns and correlate GTPBP3 levels with clinical phenotypes in mitochondrial disorders. Its specificity for the central domain ensures recognition of full-length GTPBP3 while avoiding cross-reactivity with truncated isoforms or homologous proteins. This antibody serves as a crucial reagent for advancing understanding of mitochondrial translation regulation and developing diagnostic strategies for related genetic diseases.
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