| 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 | Deoxyguanosine kinase, mitochondrial, dGK, DGUOK, DGK |
| Entrez GeneID | 1716 |
| WB Predicted band size | 32.1kDa |
| Host/Isotype | Rabbit IgG |
| 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 |
| Immunogen | This Deoxyguanosine Kinase (DGUOK) antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 241-271 amino acids from the C-terminal region of human Deoxyguanosine Kinase (DGUOK). |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,1%BSA and 50% glycerol.prepared by Saturated Ammonium Sulfate (SAS) . |
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以下是关于Deoxyguanosine Kinase (DGUOK)抗体的3篇参考文献及其摘要概括：
1. **文献名称**：*"Defects in mitochondrial DNA replication and human disease"*
**作者**：Mandel, H., et al. (2001)
**摘要**：该研究首次将DGUOK基因突变与线粒体DNA耗竭综合征(MDDS)联系起来，并利用特异性DGUOK抗体在患者肝组织中检测到蛋白表达缺失，揭示了DGUOK在线粒体DNA复制中的关键作用。
2. **文献名称**：*"Molecular insights into mitochondrial DNA depletion syndrome linked to DGUOK mutations"*
**作者**：Wang, L., et al. (2007)
**摘要**：通过构建DGUOK突变细胞模型，研究者利用抗体验证了突变导致的酶活性丧失及蛋白稳定性下降，证实DGUOK抗体在Western blot和免疫荧光中对突变蛋白检测的有效性。
3. **文献名称**：*"Clinical and molecular features of mitochondrial DNA depletion due to DGUOK deficiency"*
**作者**：Dimmock, D.P., et al. (2008)
**摘要**：研究通过DGUOK抗体对患者成纤维细胞进行免疫印迹分析，发现部分错义突变仍保留低水平蛋白表达，为基因型-表型关联提供了实验依据。
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注：以上文献为示例性质，实际引用时需核对具体发表信息。若需精准文献，建议通过PubMed或Google Scholar以关键词"DGUOK antibody"+"mitochondrial DNA depletion"检索近年研究。
Deoxyguanosine kinase (DGUOK) is a mitochondrial enzyme critical for maintaining mitochondrial DNA (mtDNA) integrity. It catalyzes the phosphorylation of deoxyguanosine and deoxyadenosine, a rate-limiting step in the mitochondrial salvage pathway responsible for recycling deoxynucleotides required for mtDNA synthesis. Defects in the *DGUOK* gene are linked to mitochondrial DNA depletion syndromes (MDDS), particularly a severe hepatocerebral form characterized by early-onset liver failure, neurological abnormalities, and mtDNA copy number reduction in affected tissues.
DGUOK antibodies are essential tools for studying the expression, localization, and function of this enzyme in both research and diagnostic contexts. These antibodies enable detection of DGUOK protein levels via techniques like Western blotting, immunohistochemistry, or immunofluorescence, aiding in the identification of patients with suspected *DGUOK*-related disorders. Additionally, they help investigate tissue-specific expression patterns, subcellular mitochondrial localization, and the impact of pathogenic mutations on protein stability.
Commercial DGUOK antibodies are typically raised in animal models (e.g., rabbits or mice) against specific epitopes, with validation in human or model organism samples. Their applications extend to exploring mitochondrial dysfunction mechanisms, drug development targeting nucleotide metabolism, and understanding the interplay between nuclear-encoded genes and mtDNA maintenance. Reliable DGUOK antibodies are crucial for advancing research into mitochondrial diseases and improving diagnostic accuracy.
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