| 纯度 | >95%SDS-PAGE. |
| 种属 | Human |
| 靶点 | PGM1 |
| Uniprot No | P36871 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-562aa |
| 氨基酸序列 | MGSSHHHHHHSSGLVPRGSHMGSMVKIVTVKTQAYQDQKPGTSGLRKRVK VFQSSANYAENFIQSIISTVEPAQRQEATLVVGGDGRFYMKEAIQLIARI AAANGIGRLVIGQNGILSTPAVSCIIRKIKAIGGIILTASHNPGGPNGDF GIKFNISNGGPAPEAITDKIFQISKTIEEYAVCPDLKVDLGVLGKQQFDL ENKFKPFTVEIVDSVEAYATMLRSIFDFSALKELLSGPNRLKIRIDAMHG VVGPYVKKILCEELGAPANSAVNCVPLEDFGGHHPDPNLTYAADLVETMK SGEHDFGAAFDGDGDRNMILGKHGFFVNPSDSVAVIAANIFSIPYFQQTG VRGFARSMPTSGALDRVASATKIALYETPTGWKFFGNLMDASKLSLCGEE SFGTGSDHIREKDGLWAVLAWLSILATRKQSVEDILKDHWQKYGRNFFTR YDYEEVEAEGANKMMKDLEALMFDRSFVGKQFSANDKVYTVEKADNFEYS DPVDGSISRNQGLRLIFTDGSRIVFRLSGTGSAGATIRLYIDSYEKDVAK INQDPQVMLAPLISIALKVSQLQERTGRTAPTVIT |
| 预测分子量 | 64 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PGM1重组蛋白的3篇参考文献示例(注：文献为虚构示例，实际文献需通过学术数据库检索)：
1. **文献名称**：*Recombinant Human PGM1: Expression, Purification, and Enzymatic Characterization*
**作者**：Smith J, et al.
**摘要**：研究报道了人源PGM1基因的克隆及在大肠杆菌中的重组表达，通过亲和层析纯化获得高活性蛋白，并验证其催化葡萄糖-1-磷酸转化为葡萄糖-6-磷酸的功能。
2. **文献名称**：*Structural Insights into PGM1 Deficiency Syndrome via Recombinant Mutant Protein Analysis*
**作者**：Lee H, et al.
**摘要**：通过重组表达PGM1常见致病突变体(如p.Phe200Ser)，分析突变对蛋白稳定性和构象的影响，揭示其与先天性糖基化障碍(CDG)的分子关联。
3. **文献名称**：*Development of a High-Yield PGM1 Production System in Pichia pastoris for Therapeutic Applications*
**作者**：Garcia R, et al.
**摘要**：优化毕赤酵母系统高效分泌表达重组PGM1.展示其在体外细胞模型中恢复糖代谢异常的治疗潜力，为罕见病药物开发提供基础。
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如需真实文献，建议通过PubMed或Google Scholar检索关键词：
**"recombinant PGM1"、"PGM1 protein expression"、"phosphoglucomutase 1 purification"**。
**Background of PGM1 Recombinant Protein**
Phosphoglucomutase 1 (PGM1) is a critical metabolic enzyme encoded by the *PGM1* gene, playing a central role in glucose homeostasis by catalyzing the interconversion of glucose-1-phosphate and glucose-6-phosphate. This reversible reaction bridges glycogen synthesis and glycolysis, making PGM1 indispensable for energy metabolism and carbohydrate processing. Mutations in *PGM1* are associated with PGM1 deficiency, a rare autosomal recessive disorder characterized by impaired glycogenolysis, hypoglycemia, and multisystemic manifestations, including muscle weakness and cardiac abnormalities.
Recombinant PGM1 protein is produced using biotechnological platforms, such as bacterial (e.g., *E. coli*) or eukaryotic (e.g., mammalian or insect cell) expression systems, to enable large-scale, high-purity enzyme production. The recombinant protein retains the enzymatic activity of native PGM1. allowing researchers to study its structure-function relationships, substrate specificity, and regulatory mechanisms. It also serves as a therapeutic candidate for enzyme replacement therapy (ERT) in PGM1-deficient patients.
Recent advances in structural biology, including X-ray crystallography and cryo-EM, have elucidated PGM1's conformational dynamics and metal ion dependency (e.g., magnesium), providing insights into catalytic mechanisms. Additionally, recombinant PGM1 is utilized in diagnostic assays to measure enzyme activity in clinical samples, aiding in the identification of genetic variants and disease diagnosis.
The development of recombinant PGM1 underscores its dual role as a research tool and a potential therapeutic, addressing both fundamental biological questions and unmet medical needs in inborn errors of metabolism. Ongoing studies focus on optimizing its stability, delivery, and efficacy in vivo to advance translational applications.
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