| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | ASAH1 |
| Uniprot No | Q13510 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 25-124aa |
| 氨基酸序列 | PPWTEDCRKSTYPPSGPTYRGAVPWYTINLDLPPYKRWHELMLDKAPMLK VIVNSLKNMINTFVPSGKVMQVVDEKLPGLLGNFPGPFEEEMKGIAAVTD |
| 预测分子量 | 37 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ASAH1重组蛋白的参考文献示例(注：内容基于模拟学术研究场景，具体文献需通过数据库验证)：
1. **标题**：Recombinant Human Acid Ceramidase (ASAH1) Expression in HEK293 Cells and Functional Characterization
**作者**：Li, X., et al.
**摘要**：研究利用HEK293细胞系统高效表达并纯化ASAH1重组蛋白，证实其具有水解神经酰胺的活性，为酶替代疗法提供基础。
2. **标题**：Structural Insights into ASAH1 Activation Mechanism via Cryo-EM Analysis
**作者**：Zhang, Y., et al.
**摘要**：通过冷冻电镜解析ASAH1重组蛋白的三维结构，揭示其底物结合域及pH依赖性激活机制，为药物设计提供结构基础。
3. **标题**：Enzyme Replacement Therapy with Recombinant ASAH1 Ameliorates Pathology in Farber Disease Mice
**作者**：Park, J.H., et al.
**摘要**：在小鼠模型中验证重组ASAH1蛋白的疗效，显著减少炎症和神经酰胺积累，证实其对Farber病的潜在治疗价值。
4. **标题**：Role of Recombinant ASAH1 in Modulating Cancer Cell Apoptosis via Ceramide Metabolism
**作者**：Smith, T., et al.
**摘要**：探讨重组ASAH1通过调节神经酰胺水平增强化疗药物诱导的肿瘤细胞凋亡，提示其作为癌症治疗辅助策略的可能性。
(注：上述文献为示例，实际引用请查询PubMed或SciHub等学术平台。)
ASAH1 recombinant protein is derived from the ASAH1 gene, which encodes the enzyme acid ceramidase (AC). This lysosomal hydrolase plays a critical role in sphingolipid metabolism by catalyzing the breakdown of ceramide into sphingosine and free fatty acids. Ceramide, a key signaling molecule, regulates cellular processes such as apoptosis, proliferation, and inflammation, while its metabolites influence diverse physiological and pathological pathways. Dysregulation of AC activity is linked to several diseases, including Farber disease (a rare lysosomal storage disorder caused by ASAH1 mutations) and spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME).
Recombinant ASAH1 protein is produced using biotechnological platforms like mammalian or bacterial expression systems, enabling large-scale synthesis for research and therapeutic applications. Its production often involves optimizing post-translational modifications (e.g., glycosylation) to ensure enzymatic activity and stability. In research, the recombinant protein serves as a tool to study ceramide metabolism, lysosomal function, and disease mechanisms. Therapeutically, it is explored for enzyme replacement therapy (ERT) in Farber disease, where deficient AC activity leads to ceramide accumulation, causing severe inflammation, joint deformities, and neurodegeneration.
Recent advances include engineered variants with improved pharmacokinetics and tissue targeting. Challenges remain in delivery across the blood-brain barrier for neurological manifestations. Beyond rare diseases, ASAH1 recombinant protein is investigated in cancer and inflammatory disorders due to ceramide's role in cell survival and immune responses. Its dual role as a metabolic enzyme and signaling modulator underscores its broad biomedical relevance, driving ongoing efforts to harness its potential in precision medicine.
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