| 纯度 | >95%SDS-PAGE. |
| 种属 | Human |
| 靶点 | GCSH |
| Uniprot No | P23434 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 49-173aa |
| 氨基酸序列 | MGSSHHHHHHSSGLVPRGSHMGSMSVRKFTEKHEWVTTENGIGTVGISNF AQEALGDVVYCSLPEVGTKLNKQDEFGALESVKAASELYSPLSGEVTEIN EALAENPGLVNKSCYEDGWLIKMTLSNPSELDELMSEEAYEKYIKSIEE |
| 预测分子量 | 16 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篇与GCSH重组蛋白相关的文献摘要信息，供参考：
1. **文献名称**：Expression and Purification of Recombinant Human Glycine Cleavage System H-Protein
**作者**：T. Fukao et al.
**摘要**：研究报道了人源GCSH蛋白在大肠杆菌中的重组表达与纯化，成功获得可溶性蛋白，并验证其参与甘氨酸裂解系统的酶活性，为遗传性高甘氨酸血症的分子机制研究提供工具。
2. **文献名称**：Structural Characterization of Recombinant GCSH Protein in Mitochondrial Disorders
**作者**：K. Okamura-Ikeda et al.
**摘要**：通过重组技术获得GCSH蛋白，解析其晶体结构，揭示其与甘氨酸裂解酶复合物其他亚基的互作机制，为线粒体代谢疾病相关突变位点的功能研究奠定基础。
3. **文献名称**：Development of a GCSH Knockout Cell Model Using CRISPR/Cas9 and Recombinant Protein Rescue
**作者**：S. Mendes et al.
**摘要**：利用CRISPR构建GCSH缺陷型细胞系，通过重组人GCSH蛋白回补实验证实其在细胞甘氨酸代谢中的关键作用，为疾病治疗策略提供体外研究平台。
(注：以上文献为模拟概括，实际文献需通过PubMed或SciFinder等数据库检索近年具体研究。)
**Background of GCSH Recombinant Protein**
Glycine cleavage system H protein (GCSH) is a mitochondrial enzyme component critical for glycine metabolism, functioning as part of the glycine cleavage system (GCS). This multienzyme complex regulates glycine catabolism by catalyzing its conversion into carbon dioxide, ammonia, and a methylene group, which contributes to one-carbon metabolism. GCSH, specifically, stabilizes the GCS complex and facilitates the lipoamide cofactor’s function in the decarboxylation process.
Mutations in the *GCSH* gene are linked to nonketotic hyperglycinemia (NKH), a rare autosomal recessive disorder characterized by glycine accumulation, leading to severe neurological impairments. Recombinant GCSH protein, produced via heterologous expression systems (e.g., *E. coli* or mammalian cells), enables detailed study of GCSH’s molecular role, disease mechanisms, and therapeutic strategies. Its production involves cloning the *GCSH* gene into expression vectors, followed by purification using affinity chromatography.
Research applications include functional assays to assess enzyme activity in NKH models, screening for pharmacological chaperones to restore mutant GCSH function, and structural studies to map interactions within the GCS complex. Additionally, recombinant GCSH serves as an antigen for antibody development, aiding diagnostic tools for NKH. Beyond disease research, it contributes to broader studies on mitochondrial metabolism, one-carbon cycle dynamics, and amino acid homeostasis.
Overall, GCSH recombinant protein is a vital tool for advancing understanding of glycine-related pathologies and exploring targeted therapies for metabolic disorders.
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