| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | D2HGDH |
| Uniprot No | Q8N465 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 14-521aa |
| 氨基酸序列 | LRGAPGAAGSWGRPVGPLARRGCCSAPGTPEVPLTRERYPVRRLPFSTVSKQDLAAFERIVPGGVVTDPEALQAPNVDWLRTLRGCSKVLLRPRTSEEVSHILRHCHERNLAVNPQGGNTGMVGGSVPVFDEIILSTARMNRVLSFHSVSGILVCQAGCVLEELSRYVEERDFIMPLDLGAKGSCHIGGNVATNAGGLRFLRYGSLHGTVLGLEVVLADGTVLDCLTSLRKDNTGYDLKQLFIGSEGTLGIITTVSILCPPKPRAVNVAFLGCPGFAEVLQTFSTCKGMLGEILSAFEFMDAVCMQLVGRHLHLASPVQESPFYVLIETSGSNAGHDAEKLGHFLEHALGSGLVTDGTMATDQRKVKMLWALRERITEALSRDGYVYKYDLSLPVERLYDIVTDLRARLGPHAKHVVGYGHLGDGNLHLNVTAEAFSPSLLAALEPHVYEWTAGQQGSVSAEHGVGFRKRDVLGYSKPPGALQLMQQLKALLDPKGILNPYKTLPSQA |
| 预测分子量 | 70.8kDa |
| 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. |
以下为假设性参考文献示例(仅供格式参考，建议通过学术数据库查询真实文献)：
1. **标题**: "Cloning and Functional Characterization of Recombinant Human D-2-Hydroxyglutarate Dehydrogenase"
**作者**: Smith A, et al.
**摘要**: 报道了人源D2HGDH基因的克隆及在大肠杆菌中的重组表达，验证了其催化D-2-羟基戊二酸转化为α-酮戊二酸的酶活性，并分析了其动力学参数。
2. **标题**: "Structural Insights into D2HGDH Deficiency Syndromes via Recombinant Protein Crystallography"
**作者**: Chen L, et al.
**摘要**: 通过重组D2HGDH蛋白的晶体结构解析，揭示了突变位点对酶活性的影响，为遗传性D-2-羟基戊二酸尿症的分子机制提供了结构基础。
3. **标题**: "D2HGDH Recombinant Protein Production in Yeast: Implications for Metabolic Disorder Therapeutics"
**作者**: García-Ruiz E, et al.
**摘要**: 开发了基于酵母系统的D2HGDH重组蛋白高效表达及纯化方法，评估了其在体外模型中对代谢异常的治疗潜力。
4. **标题**: "Role of D2HGDH in Cellular Metabolism: Evidence from RNAi and Recombinant Overexpression Studies"
**作者**: Tanaka K, et al.
**摘要**: 利用重组D2HGDH过表达及基因沉默技术，证明该酶在线粒体能量代谢和氧化应激调控中的双重作用。
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**提示**：实际文献可通过PubMed、Web of Science等平台，以"D2HGDH recombinant protein"或"D-2-hydroxyglutarate dehydrogenase"为关键词检索。关注近年《Biochimica et Biophysica Acta》《Journal of Biological Chemistry》等期刊相关研究。
D2HGDH (D-2-hydroxyglutarate dehydrogenase) is a mitochondrial enzyme encoded by the *D2HGDH* gene, primarily responsible for catalyzing the oxidation of D-2-hydroxyglutarate (D-2-HG) to 2-ketoglutarate (α-KG). This reaction plays a critical role in cellular metabolism, linking it to the tricarboxylic acid (TCA) cycle. D2HGDH functions as a flavin adenine dinucleotide (FAD)-dependent dehydrogenase and is localized in the mitochondrial matrix, where it helps maintain metabolic homeostasis by preventing the accumulation of D-2-HG, an oncometabolite associated with epigenetic dysregulation and cellular transformation.
Mutations in *D2HGDH* are linked to D-2-hydroxyglutaric aciduria (D-2-HGA), a rare neurometabolic disorder characterized by elevated D-2-HG levels, leading to developmental delays, seizures, and structural brain abnormalities. Additionally, dysregulated D2HGDH activity has been implicated in cancer, particularly in gliomas and acute myeloid leukemia (AML), where aberrant D-2-HG accumulation—often due to isocitrate dehydrogenase (IDH) mutations—drives tumorigenesis by inhibiting α-KG-dependent dioxygenases.
Recombinant D2HGDH protein is engineered for in vitro studies to elucidate its enzymatic mechanisms, substrate specificity, and interactions with metabolic pathways. Its production typically involves heterologous expression in bacterial or mammalian systems, followed by purification via affinity tags. Researchers use this tool to investigate D2HGDH's role in metabolic diseases, cancer progression, and potential therapeutic strategies targeting D-2-HG metabolism. Structural studies of recombinant D2HGDH also aid in designing inhibitors or activators to modulate its activity, offering insights into treating disorders linked to D-2-HG imbalance.
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