| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | PPGB |
| Uniprot No | P10619 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 29-480 aa |
| 活性数据 | AP DQDEIQRLPG LAKQPSFRQY SGYLKGSGSK HLHYWFVESQ KDPENSPVVL WLNGGPGCSS LDGLLTEHGP FLVQPDGVTL EYNPYSWNLI ANVLYLESPA GVGFSYSDDK FYATNDTEVA QSNFEALQDF FRLFPEYKNN KLFLTGESYA GIYIPTLAVL VMQDPSMNLQ GLAVGNGLSS YEQNDNSLVY FAYYHGLLGN RLWSSLQTHC CSQNKCNFYD NKDLECVTNL QEVARIVGNS GLNIYNLYAP CAGGVPSHFR YEKDTVVVQD LGNIFTRLPL KRMWHQALLR SGDKVRMDPP CTNTTAASTY LNNPYVRKAL NIPEQLPQWD MCNFLVNLQY RRLYRSMNSQ YLKLLSSQKY QILLYNGDVD MACNFMGDEW FVDSLNQKME VQRRPWLVKY GDSGEQIAGF VKEFSHIAFL TIKGAGHMVP TDKPLAAFTM FSRFLNKQPY |
| 分子量 | 54.4 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. |
以下是关于重组人PPGB(Protective Protein for Beta-Galactosidase)蛋白的示例参考文献，供参考：
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1. **文献名称**: *Expression and characterization of recombinant human PPGB in HEK293 cells*
**作者**: Smith, A., et al.
**摘要**: 本研究成功在HEK293细胞中表达并纯化了重组人PPGB蛋白，验证了其与β-半乳糖苷酶复合物的结合能力，证实其可恢复溶酶体中β-半乳糖苷酶的酶活性，为溶酶体贮积症治疗奠定基础。
2. **文献名称**: *Therapeutic potential of recombinant PPGB in GM1 gangliosidosis models*
**作者**: Chen, B., et al.
**摘要**: 通过动物模型实验，证明重组人PPGB蛋白可有效降解GM1神经节苷脂的异常累积，改善神经功能损伤，为GM1神经节苷脂沉积症的酶替代疗法提供依据。
3. **文献名称**: *Crystal structure of human PPGB and implications for drug design*
**作者**: Lee, J., et al.
**摘要**: 利用X射线晶体学解析PPGB的三维结构，揭示其催化位点及与底物结合的分子机制，为针对溶酶体疾病的靶向药物开发提供结构基础。
4. **文献名称**: *PPGB mutations and functional rescue by recombinant protein in lysosomal disorders*
**作者**: Patel, R., et al.
**摘要**: 系统性分析PPGB基因突变对功能的影响，并通过体外实验证实重组PPGB蛋白可补偿突变导致的酶活性缺失，提出基因治疗与酶替代联合策略。
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**注意**：以上为示例文献，实际引用时需查询PubMed、ScienceDirect等数据库获取真实文献(如涉及PPGB相关研究可替换关键词为PPT1、β-galactosidase等)。建议结合最新研究进行补充。
Recombinant human Protective Protein/Cathepsin A (PPGB), also known as lysosomal protective protein or cathepsin A, is a multifunctional enzyme encoded by the *CTSA* gene. It belongs to the serine carboxypeptidase family and plays a critical role in lysosomal homeostasis. PPGB stabilizes and activates two key lysosomal enzymes: β-galactosidase (GLB1) and neuraminidase-1 (NEU1), forming a tripartite complex essential for glycoconjugate metabolism. Deficiencies in PPGB lead to lysosomal storage disorders such as galactosialidosis, characterized by impaired degradation of glycolipids and glycoproteins, resulting in neurodegeneration, skeletal abnormalities, and organ dysfunction.
Produced via recombinant DNA technology, PPGB is typically expressed in mammalian cell systems (e.g., CHO or HEK293) to ensure proper post-translational modifications, including glycosylation critical for its stability and activity. Purification involves affinity chromatography and rigorous quality control to meet therapeutic standards. Recombinant PPGB has garnered attention for enzyme replacement therapy (ERT) in lysosomal storage diseases, particularly to address the secondary inactivation of β-galactosidase and neuraminidase in galactosialidosis. Research also explores its potential in modulating cell signaling pathways, given its role in processing bioactive peptides like endothelin-1 and angiotensin-I.
The development of recombinant PPGB highlights advancements in biopharmaceuticals to target rare genetic disorders, emphasizing precision medicine and the importance of lysosomal enzyme networks in cellular health.
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