| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/50-1/100 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/5000-1/10000 | Human,Mouse,Rat |
| Aliases | CMT2Q; AMOXAD |
| 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, Rat |
| Immunogen | Fusion protein of human DHTKD1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于DHTKD1抗体的模拟参考文献示例(实际文献需通过学术数据库查询)：
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1. **文献名称**：*DHTKD1 is essential for mitochondrial oxidative metabolism and its deficiency causes 2-aminoadipic 2-oxoadipic aciduria*
**作者**：Danhauser K, et al.
**摘要**：本研究通过免疫印迹和免疫荧光技术，利用DHTKD1特异性抗体揭示了该蛋白在线粒体中的定位。作者发现DHTKD1缺陷会导致α-酮己二酸代谢异常，与遗传性2-氨基己二酸尿症相关，证实其在线粒体能量代谢中的关键作用。
2. **文献名称**：*Structural and functional characterization of DHTKD1 in the mitochondrial lysine degradation pathway*
**作者**：Zhou Y, et al.
**摘要**：通过抗体介导的免疫沉淀和质谱分析，本研究解析了DHTKD1蛋白的复合物结构，证明其作为脱氢酶参与赖氨酸分解代谢，并验证了其突变与神经系统疾病的相关性。
3. **文献名称**：*DHTKD1 interacts with the mitochondrial electron transport chain and regulates ROS production*
**作者**：Smith J, et al.
**摘要**：利用DHTKD1抗体进行共定位实验，发现该蛋白与线粒体复合体I相互作用，调控活性氧(ROS)水平。研究提示DHTKD1可能通过氧化应激途径影响神经退行性疾病进程。
4. **文献名称**：*A novel DHTKD1 antibody validates protein expression loss in patient-derived fibroblasts*
**作者**：Garcia-Ruiz C, et al.
**摘要**：开发了一种高特异性DHTKD1单克隆抗体，用于检测患者成纤维细胞中蛋白表达缺失，为临床诊断2-氨基己二酸尿症提供了可靠工具，并验证了基因型-表型关联。
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**注意**：以上内容为模拟示例，实际文献需通过PubMed、Google Scholar等平台以“DHTKD1 antibody”或“DHTKD1 protein function”为关键词检索，并关注抗体应用场景(如Western blot、免疫组化)及疾病机制研究。
The DHTKD1 antibody is a tool used to study the dehydrogenase E1 and transketolase domain-containing 1 (DHTKD1) protein, encoded by the DHTKD1 gene. This mitochondrial protein is part of the 2-oxoacid dehydrogenase family, playing a role in lysine and tryptophan metabolism by catalyzing oxidative decarboxylation of α-ketoadipate. DHTKD1 is linked to the 2-oxoadipate dehydrogenase complex, contributing to cellular energy production and metabolite homeostasis.
Mutations in DHTKD1 are associated with 2-aminoadipic 2-oxoadipic aciduria (AMOXAD), a rare metabolic disorder characterized by elevated 2-aminoadipic and 2-oxoadipic acid levels, and possibly neuropathy. Studies also suggest a connection to Charcot-Marie-Tooth disease type 2. highlighting its role in peripheral nerve function.
The DHTKD1 antibody aids in detecting protein expression, localization, and quantification via techniques like Western blotting, immunohistochemistry, and immunofluorescence. It is valuable for exploring DHTKD1’s physiological roles, disease mechanisms, and interactions with metabolic pathways. Researchers utilize species-specific (human, mouse, rat) monoclonal or polyclonal antibodies to investigate tissue distribution, mitochondrial dysfunction, and mutation impacts. Its applications span basic research, clinical diagnostics, and therapeutic target validation in metabolic and neurodegenerative disorders.
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