| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | PDHA1 |
| Uniprot No | P08559 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 31-390aa |
| 氨基酸序列 | ANDATFEIKKCDLHRLEEGPPVTTVLTREDGLKYYRMMQTVRRMELKADQLYKQKIIRGFCHLCDGQEACCVGLEAGINPTDHLITAYRAHGFTFTRGLSVREILAELTGRKGGCAKGKGGSMHMYAKNFYGGNGIVGAQVPLGAGIALACKYNGKDEVCLTLYGDGAANQGQIFEAYNMAALWKLPCIFICENNRYGMGTSVERAAASTDYYKRGDFIPGLRVDGMDILCVREATRFAAAYCRSGKGPILMELQTYRYHGHSMSDPGVSYRTREEIQEVRSKSDPIMLLKDRMVNSNLASVEELKEIDVEVRKEIEDAAQFATADPEPPLEELGYHIYSSDPPFEVRGANQWIKFKSVS |
| 预测分子量 | 71.6 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. |
以下是关于PDHA1重组蛋白的3篇参考文献示例：
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1. **文献名称**：*"High-yield expression and purification of recombinant human PDHA1 in Escherichia coli for functional studies"*
**作者**：Smith A, et al.
**摘要**：本研究报道了利用大肠杆菌表达系统高效表达人源PDHA1重组蛋白的优化方法。通过构建His标签融合蛋白，采用镍柱亲和层析和离子交换色谱纯化，获得高纯度蛋白。酶活性实验证实重组PDHA1具有丙酮酸脱氢酶活性，为后续功能研究奠定基础。
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2. **文献名称**：*"Structural insights into PDHA1 mutations associated with pyruvate dehydrogenase complex deficiency"*
**作者**：Brown C, et al.
**摘要**：通过X射线晶体学解析了PDHA1野生型和两种致病突变体(R234C、G298S)的蛋白结构，揭示了突变对酶活性中心构象的影响。实验表明突变导致辅酶TPP结合能力下降，阐明了其引发丙酮酸脱氢酶缺乏症的分子机制。
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3. **文献名称**：*"Functional rescue of PDHA1-deficient cells by recombinant protein delivery"*
**作者**：Lee J, et al.
**摘要**：研究开发了基于脂质体的重组PDHA1蛋白递送系统，成功恢复PDHA1缺陷型细胞的线粒体代谢功能。细胞实验显示，递送的蛋白定位于线粒体并显著提高丙酮酸氧化速率，为酶替代疗法提供了潜在策略。
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**注**：以上文献为示例，实际引用时需核实真实来源。建议通过PubMed或Web of Science以“PDHA1 recombinant protein”为关键词检索最新研究。
PDHA1 (Pyruvate Dehydrogenase E1 Alpha 1 Subunit) is a critical enzyme component of the mitochondrial pyruvate dehydrogenase complex (PDC), which plays a central role in cellular energy metabolism. This complex catalyzes the oxidative decarboxylation of pyruvate to acetyl-CoA, linking glycolysis to the tricarboxylic acid (TCA) cycle. PDHA1 encodes the α-subunit of the E1 heterotetramer (α2β2), responsible for the decarboxylation of pyruvate and the subsequent transfer of the acetyl group to the E2 subunit. Its activity is tightly regulated by phosphorylation/dephosphorylation mechanisms, which modulate PDC function in response to nutrient availability and cellular energy demands.
Recombinant PDHA1 protein is engineered using molecular cloning techniques to express the human PDHA1 gene in heterologous systems, such as *E. coli*, yeast, or mammalian cell lines. This allows large-scale production of purified, functional PDHA1 for research and therapeutic applications. The recombinant protein retains enzymatic activity and structural integrity, enabling studies on PDC regulation, substrate specificity, and interactions with regulatory kinases/phosphatases. It is particularly valuable for investigating PDHA1 mutations linked to pyruvate dehydrogenase deficiency (PDHD), a rare metabolic disorder characterized by lactic acidosis, neurological impairment, and developmental delays. By analyzing recombinant variants, researchers can elucidate genotype-phenotype correlations and test potential therapies. Additionally, PDHA1 recombinant protein serves as an antigen for antibody development, a tool for drug screening, and a reference standard in diagnostic assays. Its production supports advances in understanding mitochondrial dysfunction, metabolic diseases, and cancer metabolism, where altered PDC activity is implicated in the Warburg effect.
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