| 纯度 | > 90 % SDS-PAGE. |
| 种属 | Human |
| 靶点 | ASS1 |
| Uniprot No | P00966 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-412aa |
| 氨基酸序列 | MGSSHHHHHH SSGLVPRGSH MSSKGSVVLA YSGGLDTSCI LVWLKEQGYD VIAYLANIGQ KEDFEEARKK ALKLGAKKVF IEDVSREFVEEFIWPAIQSS ALYEDRYLLG TSLARPCIAR KQVEIAQREG AKYVSHGATG KGNDQVRFEL SCYSLAPQIK VIAPWRMPEF YNRFKGRNDL MEYAKQHGIP IPVTPKNPWS MDENLMHISY EAGILENPKN QAPPGLYTKT QDPAKAPNTP DILEIEFKKG VPVKVTNVKD GTTHQTSLELFMYLNEVAGK HGVGRIDIVE NRFIGMKSRG IYETPAGTIL YHAHLDIEAF TMDREVRKIK QGLGLKFAEL VYTGFWHSPE CEFVRHCIAK SQERVEGKVQ VSVLKGQVYI LGRESPLSLY NEELVSMNVQ GDYEPTDATG FININSLRLK EYHRLQSKVT AK |
| 预测分子量 | 49 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. |
以下是与ASS1重组蛋白相关的参考文献示例：
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1. **文献名称**：*Recombinant expression and functional characterization of human argininosuccinate synthase (ASS1)*
**作者**：Yang X, et al.
**摘要**：研究报道了人源ASS1基因在大肠杆菌中的重组表达与纯化，验证了其酶活性，并分析了ASS1突变体在尿素循环缺陷症中的功能影响。
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2. **文献名称**：*Structural insights into ASS1 deficiency using recombinant protein models*
**作者**：Smith J, et al.
**摘要**：通过X射线晶体学解析重组ASS1蛋白的结构，揭示了其底物结合域的关键氨基酸残基，为ASS1缺陷相关疾病的治疗提供分子基础。
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3. **文献名称**：*ASS1 recombinant protein therapy ameliorates metabolic dysfunction in a murine model*
**作者**：Lee H, et al.
**摘要**：在ASS1缺陷小鼠模型中，重组ASS1蛋白通过静脉注射显著恢复尿素循环功能，降低血浆氨水平，证明其作为酶替代疗法的潜力。
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4. **文献名称**：*High-yield production of bioactive human ASS1 in mammalian cell systems*
**作者**：Garcia-Ruiz C, et al.
**摘要**：利用哺乳动物细胞表达系统优化ASS1重组蛋白的生产工艺，获得高纯度、具有天然构象的ASS1.适用于体外疾病机制研究和药物筛选。
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注：以上文献信息为模拟示例，实际引用时需核实具体来源及细节。
**Background of Recombinant ASS1 Protein**
Argininosuccinate synthase 1 (ASS1) is a critical enzyme in the urea cycle, catalyzing the condensation of citrulline and aspartate to form argininosuccinate, a key step in ammonia detoxification and arginine biosynthesis. Mutations in the *ASS1* gene disrupt the urea cycle, leading to citrullinemia type I, a life-threatening metabolic disorder characterized by hyperammonemia and neurological complications. Beyond its metabolic role, ASS1 has gained attention in cancer biology, as its downregulation in certain tumors (e.g., hepatocellular carcinoma, melanoma) is linked to metabolic reprogramming, enabling cancer cells to exploit extracellular arginine for growth—a vulnerability targeted by arginine-depleting therapies.
Recombinant ASS1 protein is engineered using biotechnological platforms (e.g., *E. coli*, mammalian, or insect cell systems) to produce functional enzyme for research and therapeutic applications. Its production involves cloning the *ASS1* cDNA into expression vectors, followed by purification via affinity chromatography (e.g., His-tag systems). The recombinant protein retains catalytic activity, enabling studies on enzyme kinetics, substrate specificity, and inhibitor screening. It also serves as a reference standard in diagnostic assays for citrullinemia and as a potential therapeutic agent in enzyme replacement strategies.
Recent advances focus on optimizing recombinant ASS1 stability and delivery, particularly for crossing biological barriers like the blood-brain barrier to address neurological symptoms in urea cycle disorders. Additionally, structural studies using recombinant ASS1 have elucidated mechanisms of pathogenic mutations and guided drug design. Overall, recombinant ASS1 is a vital tool for understanding urea cycle biology, developing diagnostics, and exploring novel treatments for metabolic diseases and cancer.
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