| 纯度 | >95%SDS-PAGE. |
| 种属 | Human |
| 靶点 | HEXB |
| Uniprot No | P07686 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 43-556aa |
| 氨基酸序列 | ARAPSVSAKP GPALWPLPLS VKMTPNLLHL APENFYISHS PNSTAGPSCT LLEEAFRRYH GYIFGFYKWH HEPAEFQAKT QVQQLLVSIT LQSECDAFPN ISSDESYTLL VKEPVAVLKA NRVWGALRGL ETFSQLVYQD SYGTFTINES TIIDSPRFSH RGILIDTSRH YLPVKIILKT LDAMAFNKFN VLHWHIVDDQ SFPYQSITFP ELSNKGSYSL SHVYTPNDVR MVIEYARLRG IRVLPEFDTP GHTLSWGKGQ KDLLTPCYSR QNKLDSFGPI NPTLNTTYSF LTTFFKEISE VFPDQFIHLG GDEVEFKCWE SNPKIQDFMR QKGFGTDFKK LESFYIQKVL DIIATINKGS IVWQEVFDDK AKLAPGTIVE VWKDSAYPEE LSRVTASGFP VILSAPWYLD LISYGQDWRK YYKVEPLDFG GTQKQKQLFI GGEACLWGEY VDATNLTPRL WPRASAVGER LWSSKDVRDM DDAYDRLTRH RCRMVERGIA AQPLYAGYCN HENM |
| 预测分子量 | 60 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HEXB重组蛋白的3篇示例文献(注：文献信息为模拟示例，非真实存在，仅供格式参考)：
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1. **文献名称**：*Production and characterization of recombinant human β-hexosaminidase B in CHO cells for therapeutic applications*
**作者**：Smith A, et al.
**摘要**：研究团队在CHO细胞中成功表达了具有生物活性的重组人HEXB蛋白，并验证其酶动力学特性。该蛋白可有效降解GM2神经节苷脂，为桑德霍夫病的酶替代疗法提供了潜在候选药物。
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2. **文献名称**：*Crystal structure of recombinant HEXB subunit reveals key residues for substrate binding*
**作者**：Tanaka K, et al.
**摘要**：通过X射线晶体学解析了重组HEXB蛋白的三维结构，揭示了其与底物结合的活性位点及关键氨基酸残基，为理解致病性突变(如c.1612C>T)导致的酶功能缺陷提供了结构基础。
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3. **文献名称**：*AAV-mediated HEXB gene delivery rescues motor deficits in a murine model of Sandhoff disease*
**作者**：Chen L, et al.
**摘要**：利用腺相关病毒(AAV)载体递送重组HEXB基因至桑德霍夫病模型小鼠，显著恢复脑组织中的β-己糖胺酶活性，并改善运动功能障碍和神经元病理特征，证实基因疗法的可行性。
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注：如需真实文献，建议在PubMed或Web of Science中以“HEXB recombinant protein”、“HEXB gene therapy”等关键词检索近年研究。
**Background of HEXB Recombinant Protein**
HEXB, or beta-hexosaminidase subunit beta, is a critical lysosomal enzyme encoded by the *HEXB* gene. It forms a heterodimer with the alpha subunit (HEXA) to constitute β-hexosaminidase A, which hydrolyzes GM2 gangliosides and other glycoconjugates in lysosomes. Deficiencies in HEXB result in impaired degradation of these substrates, leading to lysosomal storage disorders such as Sandhoff disease—a severe neurodegenerative condition characterized by GM2 accumulation, progressive neurological decline, and early mortality.
Recombinant HEXB protein is produced via genetic engineering, typically using mammalian expression systems (e.g., CHO or HEK293 cells) to ensure proper post-translational modifications and enzymatic activity. This engineered protein serves as a vital tool for studying HEXB function, disease mechanisms, and therapeutic strategies. Purification techniques like affinity chromatography yield high-purity HEXB for research or preclinical applications.
Therapeutic interest in recombinant HEXB centers on enzyme replacement therapy (ERT) and gene therapy for Sandhoff disease. However, challenges such as blood-brain barrier penetration limit ERT efficacy for neurological symptoms. Innovative approaches, including intrathecal enzyme delivery or viral vector-mediated gene transfer, are under investigation. Additionally, recombinant HEXB is used to generate disease models, screen chaperone molecules, and validate gene-editing therapies like CRISPR-Cas9.
Current research also explores combining HEXB with HEXA to restore hexosaminidase activity comprehensively. Despite progress, optimizing bioavailability, stability, and delivery remains critical for clinical translation. Recombinant HEXB thus represents a cornerstone in both understanding lysosomal biology and developing targeted interventions for GM2 gangliosidoses.
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