| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | PDHb |
| Uniprot No | P11177 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 31-359aa |
| 氨基酸序列 | LQVTVRDAINQGMDEELERDEKVFLLGEEVAQYDGAYKVSRGLWKKYGDKRIIDTPISEMGFAGIAVGAAMAGLRPICEFMTFNFSMQAIDQVINSAAKTYYMSGGLQPVPIVFRGPNGASAGVAAQHSQCFAAWYGHCPGLKVVSPWNSEDAKGLIKSAIRDNNPVVVLENELMYGVPFEFPPEAQSKDFLIPIGKAKIERQGTHITVVSHSRPVGHCLEAAAVLSKEGVECEVINMRTIRPMDMETIEASVMKTNHLVTVEGGWPQFGVGAEICARIMEGPAFNFLDAPAVRVTGADVPMPYAKILEDNSIPQVKDIIFAIKKTLNI |
| 预测分子量 | 41.9 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3-4条关于PDHb(丙酮酸脱氢酶复合体β亚基)重组蛋白的模拟参考文献示例(实际文献需根据具体研究检索)：
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1. **文献名称**：*"Recombinant expression and functional characterization of human pyruvate dehydrogenase beta subunit (PDHB) in Escherichia coli"*
**作者**：Kato, M., et al.
**摘要**：研究通过大肠杆菌表达系统成功重组表达了人源PDHb蛋白，并验证了其在丙酮酸脱氢酶复合体(PDH)中的催化活性。实验表明，重组PDHb能够恢复PDH复合体缺陷型细胞的代谢功能，为研究PDH相关疾病(如乳酸酸中毒)提供了工具。
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2. **文献名称**：*"Structural insights into the role of PDHB mutations in metabolic disorders"*
**作者**：Patel, R., & Smith, J.
**摘要**：通过重组PDHb蛋白的结晶和突变分析，揭示了多个与遗传性PDH缺乏症相关的突变位点(如p.Arg127Gln)如何破坏蛋白结构稳定性，导致酶复合体功能丧失，为靶向治疗提供了结构基础。
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3. **文献名称**：*"Optimization of PDHB recombinant protein purification for enzymatic assays"*
**作者**：Thorburn, D.R., et al.
**摘要**：开发了一种高效的重组PDHb蛋白纯化方案，利用亲和层析和尺寸排阻色谱技术获得高纯度蛋白，并验证了其在体外酶活测定中的应用，为高通量药物筛选奠定了基础。
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4. **文献名称**：*"Functional complementation of mitochondrial PDHB deficiency using recombinant adenovirus vectors"*
**作者**：Zhang, Y., et al.
**摘要**：通过腺病毒载体递送重组PDHb蛋白至PDHB缺陷的细胞模型，恢复了线粒体丙酮酸代谢通量，证明基因治疗策略在纠正PDH功能障碍中的潜在应用价值。
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**注意**：以上文献为示例性内容，实际研究中请通过PubMed、Google Scholar等平台检索真实文献(关键词：PDHB, recombinant protein, pyruvate dehydrogenase)。
**Background of Recombinant PDHb Protein**
Pyruvate dehydrogenase E1 component subunit beta (PDHb), encoded by the *PDHB* gene, is a critical metabolic enzyme involved in glucose homeostasis. It serves as a subunit of the pyruvate dehydrogenase complex (PDC), a mitochondrial multienzyme system that catalyzes the irreversible conversion of pyruvate to acetyl-CoA, linking glycolysis to the tricarboxylic acid (TCA) cycle. This reaction is essential for cellular energy production, as acetyl-CoA fuels ATP generation and biosynthetic pathways. Structurally, the PDH complex comprises three core components: E1 (pyruvate decarboxylase), E2 (dihydrolipoamide acetyltransferase), and E3 (dihydrolipoamide dehydrogenase). The E1 component, a heterotetramer of PDHα and PDHb subunits, plays a pivotal role in decarboxylating pyruvate and transferring the acetyl group to E2.
Dysregulation of PDHb is linked to metabolic disorders, particularly PDH deficiency, a rare genetic condition characterized by lactic acidosis and neurological dysfunction. Mutations in *PDHB* disrupt enzyme activity, impairing glucose oxidation and leading to energy deficits in high-demand tissues like the brain. To study its function and develop therapeutic strategies, recombinant PDHb protein is produced using heterologous expression systems, such as *E. coli* or mammalian cell cultures. This involves cloning the *PDHB* gene into expression vectors, followed by purification via affinity chromatography or tag-based methods.
Recombinant PDHb enables detailed biochemical analyses, including enzymatic activity assays, structural studies (e.g., X-ray crystallography), and investigations into mutation impacts. It also aids in drug screening for metabolic diseases and serves as an antigen for antibody development. Advances in recombinant technology have enhanced the accessibility of high-purity, functional PDHb, accelerating research into mitochondrial disorders and potential gene therapies. Overall, recombinant PDHb is a vital tool for unraveling metabolic mechanisms and addressing PDH-related pathologies.
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