| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | HEXA |
| Uniprot No | P06865 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-529aa |
| 氨基酸序列 | MTSSRLWFSLLLAAAFAGRATALWPWPQNFQTSDQRYVLYPNNFQFQYDV S SAAQPGCSVLDEAFQRYRDLLFGSGSWPRPYLTGKRHTLEKNVLVVS VVTPGCNQLPTLESVENYTLTINDDQCLLLSETVWGALRGLETFSQLVWK SAEGTFFINKTEIEDFPRFPHRGLLLDTSRHYLPLSSILDTLDVMAYNKL NVFHWHLVDDPSFPYESFTFPELMRKGSYNPVTHIYTAQDVKEVIEYARL RGIRVLAEFDTPGHTLSWGPGIPGLLTPCYSGSEPSGTFGPVNPSLNNTY EFMSTFFLEVSSVFPDFYLHLGGDEVDFTCWKSNPEIQDFMRKKGFGEDF KQLESFYIQTLLDIVSSYGKGYVVWQEVFDNKVKIQPDTIIQVWREDIPV NYMKELELVTKAGFRALLSAPWYLNRISYGPDWKDFYIVEPLAFEGTPEQ KALVIGGEACMWGEYVDNTNLVPRLWPRAGAVAERLWSNKLTSDLTFAYE RLSHFRCELLRRGVQAQPLNVGFCEQEFEQTDYKDDDDKHHHHHH |
| 预测分子量 | 63 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. |
以下为3篇与HEXA重组蛋白相关的文献摘要概括：
1. **文献名称**: "Expression and purification of recombinant human β-hexosaminidase A in mammalian cells"
**作者**: Maegawa GH et al.
**摘要**: 该研究通过哺乳动物细胞表达系统(CHO细胞)成功生产了具有酶活性的HEXA重组蛋白，并优化了纯化流程，证实其可用于体外酶活性检测及溶酶体贮积症治疗研究。
2. **文献名称**: "Enzymatic characterization of HEXA variants using recombinant protein models"
**作者**: Tropak MB et al.
**摘要**: 通过大肠杆菌表达系统制备了多种HEXA突变体重组蛋白，分析了不同突变对酶结构稳定性和催化功能的影响，为泰-萨克斯病的分子机制研究提供工具。
3. **文献名称**: "Gene therapy using HEXA/B recombinant enzyme fusion for Tay-Sachs disease"
**作者**: Osher EC et al.
**摘要**: 构建了HEXA与HEXB亚基的融合重组蛋白，验证其在体外细胞模型中的协同酶活性恢复能力，探索其在基因治疗中替代缺陷酶的潜在应用价值。
注：以上文献信息为示例性概括，实际文献检索建议通过PubMed、Web of Science等数据库以关键词“HEXA recombinant protein”或“hexosaminidase A expression”获取最新研究。
HEXA recombinant protein is derived from the HEXA gene, which encodes the α-subunit of the lysosomal enzyme β-hexosaminidase A. This enzyme plays a critical role in breaking down GM2 gangliosides, a class of complex lipids essential for neuronal function. Mutations in HEXA disrupt β-hexosaminidase A activity, leading to GM2 accumulation in lysosomes. This underlies Tay-Sachs disease, a fatal neurodegenerative disorder characterized by progressive motor and cognitive decline, typically manifesting in infancy.
Recombinant HEXA protein is produced using biotechnological platforms, such as bacterial, yeast, or mammalian cell expression systems, to enable large-scale synthesis. Its production often involves optimizing codon usage, purification tags, and post-translational modifications (e.g., glycosylation in mammalian systems) to enhance stability and enzymatic activity.
Research on HEXA recombinant protein focuses on therapeutic applications, particularly enzyme replacement therapy (ERT) and gene therapy. While ERT faces challenges like enzyme instability and poor blood-brain barrier penetration, studies explore delivery methods such as intrathecal administration or nanoparticle carriers. Additionally, recombinant HEXA serves as a tool for studying disease mechanisms, screening potential drugs, and developing gene-editing strategies (e.g., CRISPR-Cas9) to correct HEXA mutations.
Recent advances include engineered HEXA variants with improved lysosomal targeting or modified glycosylation patterns to enhance cellular uptake. However, obstacles remain in achieving sustained enzyme activity and minimizing immune responses. Despite these hurdles, HEXA recombinant protein remains pivotal in advancing therapeutic interventions and understanding lysosomal storage disorders. Its development exemplifies the intersection of molecular biology and translational medicine, offering hope for treating previously incurable genetic diseases.
×
