| 纯度 | >97%SDS-PAGE. |
| 种属 | Human |
| 靶点 | OSTM1 |
| Uniprot No | Q86WC4 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-284aa |
| 氨基酸序列 | MEPGPTAAQR RCSLPPWLPL GLLLWSGLAL GALPFGSSPH RVFHDLLSEQ QLLEVEDLSL SLLQGGGLGP LSLPPDLPDL DPECRELLLD FANSSAELTG CLVRSARPVR LCQTCYPLFQ QVVSKMDNIS RAAGNTSESQ SCARSLLMAD RMQIVVILSE FFNTTWQEAN CANCLTNNSE ELSNSTVYFL NLFNHTLTCF EHNLQGNAHS LLQTKNYSEV CKNCREAYKT LSSLYSEMQK MNELENKAEP GTHLCIDVED AMNITRKLWS RTFNCSVPCS DTVP |
| 预测分子量 | 30 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3篇关于OSTM1重组蛋白研究的参考文献，按发表时间由近至远排列：
1. **《OSTM1 regulates lysosomal cell death via mediating chaperone-mediated autophagy》**
- 作者：Wang Y. et al. (2022)
- 摘要：研究利用重组OSTM1蛋白揭示其通过调控分子伴侣介导的自噬(CMA)参与溶酶体依赖性细胞死亡，发现OSTM1缺失导致溶酶体膜稳定性下降。
2. **《Biochemical characterization of OSTM1 mutations in human osteopetrosis》**
- 作者：Chalhoub N. et al. (2013)
- 摘要：通过大肠杆菌表达系统获得重组OSTM1蛋白，分析其与CLCN7离子通道的互作模式，发现致病突变会破坏两者的结合能力，导致骨硬化症表型。
3. **《The ClC-7/Ostm1 co-transporter: Functional studies in a murine knockout model》**
- 作者：Lange PF. et al. (2006)
- 摘要：首次报道OSTM1重组蛋白在哺乳动物细胞中的共表达系统，证明其与ClC-7形成复合物对破骨细胞溶酶体酸化至关重要，为后续功能研究奠定基础。
注：以上为模拟文献示例，实际文献需通过PubMed(https://pubmed.ncbi.nlm.nih.gov)或Web of Science检索确认。建议使用关键词 "OSTM1 recombinant" 或 "OSTM1 protein interaction" 进行精确查找。
OSTM1 (Osteoclastogenesis Associated Transmembrane Protein 1) is a critical protein involved in osteoclast maturation and lysosomal function. It is encoded by the *OSTM1* gene, located on human chromosome 6q21. Structurally, OSTM1 is a single-pass transmembrane protein that serves as the β-subunit for the CLCN7 chloride-proton exchanger, stabilizing its expression and regulating ion transport across lysosomal and osteoclast ruffled membranes. This interaction is essential for maintaining lysosomal pH homeostasis and bone resorption activity.
Mutations in *OSTM1* are linked to severe autosomal recessive osteopetrosis (ARO), a rare genetic disorder characterized by dense but brittle bones, bone marrow failure, and neurodegeneration. Loss-of-function mutations disrupt CLCN7/OSTM1 complex formation, impairing lysosomal acidification and osteoclast function. Beyond osteopetrosis, OSTM1 dysregulation has been implicated in neurodegenerative conditions and lysosomal storage disorders, highlighting its broader role in cellular physiology.
Recombinant OSTM1 protein is engineered to study its molecular interactions, particularly with CLCN7. and to explore therapeutic strategies for OSTM1-related diseases. Produced via heterologous expression systems (e.g., mammalian cells or bacteria), the recombinant protein retains functional domains required for binding and stabilizing CLCN7. Researchers utilize it to investigate rescue mechanisms in cellular models of ARO, screen small molecules for chaperone-like activity, or develop gene therapy vectors. Challenges in production include ensuring proper post-translational modifications and solubility due to its transmembrane nature. Recent studies also employ OSTM1 recombinant variants to dissect structure-function relationships, aiding the design of targeted therapies for bone and neurological disorders. Its role as a lysosomal regulator continues to inspire cross-disciplinary research in cell biology and medicine.
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