| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | BCS1L |
| Uniprot No | Q9Y276 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-419aa |
| 氨基酸序列 | MPLSDFILALKDNPYFGAGFGLVGVGTALALARKGVQLGLVAFRRHYMITLEVPARDRSYAWLLSWLTRHSTRTQHLSVETSYLQHESGRISTKFEFVPSPGNHFIWYRGKWIRVERSREMQMIDLQTGTPWESVTFTALGTDRKVFFNILEEARELALQQEEGKTVMYTAVGSEWRPFGYPRRRRPLNSVVLQQGLADRIVRDVQEFIDNPKWYTDRGIPYRRGYLLYGPPGCGKSSFITALAGELEHSICLLSLTDSSLSDDRLNHLLSVAPQQSLVLLEDVDAAFLSRDLAVENPVKYQGLGRLTFSGLLNALDGVASTEARIVFMTTNHVDRLDPALIRPGRVDLKEYVGYCSHWQLTQMFQRFYPGQAPSLAENFAEHVLRATNQISPAQVQGYFMLYKNDPVGAIHNAESLRR |
| 分子量 | 73.9 kDa |
| 蛋白标签 | GST-tag at N-terminal |
| 缓冲液 | 冻干粉 |
| 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BCS1L的三篇代表性文献摘要(作者与内容为综合描述，非真实文献)：
1. **标题**: "Structural insights into BCS1L-mediated assembly of mitochondrial complex III"
**作者**: Basu, S., et al. (2016)
**摘要**: 通过冷冻电镜解析BCS1L蛋白的三维结构，揭示其作为线粒体AAA-ATP酶家族成员如何促进细胞色素b和Rieske蛋白的组装，维持复合体III的功能完整性。
2. **标题**: "BCS1L mutations cause mitochondrial encephalopathy through impaired respiratory chain biogenesis"
**作者**: Fernández-Vizarra, E., et al. (2007)
**摘要**: 研究BCS1L基因突变导致新生儿线粒体脑病的机制，发现突变会破坏复合体III的组装过程，导致呼吸链功能障碍和ATP合成减少。
3. **标题**: "BCS1L interacts with prohibitins to regulate reactive oxygen species in mitochondria"
**作者**: Vukotic, M., et al. (2017)
**摘要**: 揭示BCS1L与线粒体内膜蛋白prohibitin的互作关系，提出其通过调控ROS稳态影响细胞氧化应激响应，为相关代谢疾病提供新机制解释。
4. **标题**: "Recombinant BCS1L protein rescues respiratory defects in patient-derived cells"
**作者**: Tucker, E.J., et al. (2013)
**摘要**: 利用重组表达的BCS1L蛋白在患者细胞中进行功能回补实验，证明外源性BCS1L可修复复合体III组装缺陷，为基因治疗提供实验依据。
*注：以上文献信息为示例性整合内容，实际引用需核对真实论文数据。*
BCS1L (BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone) is a critical AAA+ ATPase chaperone protein in human mitochondria, essential for the proper assembly of respiratory chain Complex III (cytochrome bc₁ complex). Located in the inner mitochondrial membrane, BCS1L facilitates the insertion of the Rieske iron-sulfur protein (ISP) subunit into Complex III, a vital step for electron transport chain functionality and oxidative phosphorylation. Mutations in the BCS1L gene are linked to severe mitochondrial disorders, including GRACILE syndrome, Björnstad syndrome, and encephalopathy, reflecting its role in maintaining mitochondrial proteostasis.
Recombinant BCS1L is engineered to study its ATP-dependent chaperone mechanisms, structural dynamics, and pathogenic mutation effects. Its hexameric structure and conformational changes during substrate remodeling are key research focuses. Recombinant variants enable biochemical assays, structural analysis (e.g., cryo-EM), and drug screening for mitochondrial pathologies. Current studies emphasize its interaction with co-chaperones like Hsp70/HSPA9 and substrate recognition patterns. Understanding BCS1L’s function aids in elucidating mitochondrial disease pathways and developing targeted therapies, particularly for disorders caused by Complex III deficiencies.
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