| 纯度 | >85%SDS-PAGE. |
| 种属 | Human |
| 靶点 | PDHX |
| Uniprot No | O00330 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 54-501aa |
| 氨基酸序列 | MGSSHHHHHH SSGLVPRGSH MGSGDPIKIL MPSLSPTMEE GNIVKWLKKE GEAVSAGDAL CEIETDKAVV TLDASDDGIL AKIVVEEGSK NIRLGSLIGL IVEEGEDWKH VEIPKDVGPP PPVSKPSEPR PSPEPQISIP VKKEHIPGTL RFRLSPAARN ILEKHSLDAS QGTATGPRGI FTKEDALKLV QLKQTGKITE SRPTPAPTAT PTAPSPLQAT AGPSYPRPVI PPVSTPGQPN AVGTFTEIPA SNIRRVIAKR LTESKSTVPH AYATADCDLG AVLKVRQDLV KDDIKVSVND FIIKAAAVTL KQMPDVNVSW DGEGPKQLPF IDISVAVATD KGLLTPIIKD AAAKGIQEIA DSVKALSKKA RDGKLLPEEY QGGSFSISNL GMFGIDEFTA VINPPQACIL AVGRFRPVLK LTEDEEGNAK LQQRQLITVT MSSDSRVVDD ELATRFLKSF KANLENPIRL A |
| 预测分子量 | 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. |
以下是3条关于PDHX重组蛋白的虚构参考文献示例(基于常见研究方向总结，非真实文献)：
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1. **文献名称**: *Expression and Purification of Recombinant Human PDHX Protein in E. coli*
**作者**: Smith J, Lee K, Zhang Y
**摘要**: 本研究成功构建了人源PDHX基因的原核表达载体，利用大肠杆菌系统高效表达可溶性PDHX重组蛋白，并通过亲和层析和离子交换层析实现高纯度制备，为后续功能研究奠定基础。
2. **文献名称**: *Structural Insights into PDHX Function in the Pyruvate Dehydrogenase Complex*
**作者**: Chen L, Wang H, Gupta R
**摘要**: 通过X射线晶体学解析PDHX重组蛋白的三维结构，揭示了其与丙酮酸脱氢酶复合体其他亚基(如E1、E2)的相互作用界面，阐明了PDHX在复合体组装和催化中的调控机制。
3. **文献名称**: *PDHX Deficiency: Functional Rescue by Recombinant Protein Therapy in a Mouse Model*
**作者**: Müller S, Rossi A, Park S
**摘要**: 研究利用重组PDHX蛋白在PDHX缺陷小鼠模型中开展治疗实验，证实外源性蛋白可部分恢复丙酮酸脱氢酶活性，改善代谢紊乱，提示重组PDHX在遗传性代谢疾病中的潜在应用价值。
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注：以上文献为示例性内容，实际文献需通过PubMed或学术数据库(如Google Scholar)检索关键词“PDHX recombinant protein”获取。
The PDHX gene encodes the E3-binding protein component of the pyruvate dehydrogenase (PDH) complex, a critical mitochondrial enzyme system responsible for converting pyruvate to acetyl-CoA during cellular glucose oxidation. This reaction links glycolysis to the citric acid cycle, making the PDH complex indispensable for energy production. The PDHX protein stabilizes the structural integrity of the PDH complex by anchoring the E3 (dihydrolipoamide dehydrogenase) subunit to the core E1/E2 subunits. Defects in PDHX are associated with PDH complex deficiency, a rare metabolic disorder characterized by lactic acidosis, neurological impairment, and developmental delays.
Recombinant PDHX protein is engineered using heterologous expression systems (e.g., E. coli, mammalian cells) to produce a purified, functional form of the protein for research and therapeutic applications. Its production typically involves cloning the PDHX cDNA into expression vectors, followed by affinity chromatography for purification. The recombinant protein retains native structural and functional properties, enabling studies on PDH complex assembly, enzyme kinetics, and molecular interactions.
Researchers utilize recombinant PDHX to investigate disease mechanisms, screen potential therapeutics for PDH deficiency, and develop diagnostic tools. It also serves as an antigen in autoantibody detection assays for autoimmune disorders linked to PDH complex dysfunction. Additionally, recombinant PDHX aids in structural studies (e.g., X-ray crystallography, cryo-EM) to map binding domains and elucidate regulatory mechanisms. Recent advancements in protein engineering have improved its stability and solubility, enhancing its utility in high-throughput assays and gene therapy vector development. Quality control measures, including SDS-PAGE, Western blotting, and enzymatic activity assays, ensure batch consistency for experimental reproducibility.
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