| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | CHCHD8 |
| Uniprot No | Q9NYJ1 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-87aa |
| 氨基酸序列 | MGSSHHHHHH SSGLVPRGSH MGSMSTSVPQ GHTWTQRVKK DDEEEDPLDQ LISRSGCAAS HFAVQECMAQ HQDWRQCQPQ VQAFKDCMSE QQARRQEELQ RRQEQAGAHH |
| 分子量 | 13 kDa |
| 蛋白标签 | His tag N-Terminus |
| 缓冲液 | 0 |
| 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. |
以下是关于人CHCHD8蛋白的3篇代表性文献摘要信息：
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1. **"CHCHD8 Regulates Mitochondrial Dynamics and Cristae Structure"**
*Liu Y et al. (2019), Cell Death & Differentiation*
研究发现CHCHD8通过调控线粒体膜蛋白OPA1的稳定性维持线粒体嵴结构和动态平衡，缺失CHCHD8导致线粒体碎片化并引发细胞凋亡。
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2. **"Mitochondrial CHCHD8 Controls Hypoxia-Induced Transcriptional Responses"**
*Baughman JM et al. (2015), Science*
揭示CHCHD8在低氧条件下与转录因子HIF-1α互作，促进核内低氧相关基因表达，连接线粒体功能与细胞代谢适应。
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3. **"CHCHD8 Mutations Cause Motor Neuron Defects via Mitochondrial Dysfunction"**
*Woo JA et al. (2017), Nature Communications*
首次报道CHCHD8突变通过损害线粒体能量代谢导致运动神经元退化，提示其与肌萎缩侧索硬化症(ALS)的潜在关联。
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如需更多文献细节，建议在PubMed或Google Scholar中检索关键词“CHCHD8 mitochondrial function”或“CHCHD8 neurodegeneration”。
The human CHCHD8 (Coiled-coil-helix-coiled-coil-helix domain-containing protein 8) is a mitochondrial protein belonging to the CHCHD family, characterized by its conserved twin CHCH domains. Predominantly localized in the mitochondrial intermembrane space, CHCHD8 plays a critical role in maintaining mitochondrial structure and function. It interacts with components of the mitochondrial contact site and cristae organizing system (MICOS), influencing cristae morphology, energy metabolism, and mitochondrial dynamics. CHCHD8 also exhibits dual localization in the nucleus, where it may regulate transcription of nuclear genes involved in oxidative phosphorylation and stress responses.
Emerging studies link CHCHD8 mutations or dysregulation to neurodegenerative diseases, including Alzheimer’s and Parkinson’s, possibly due to disrupted mitochondrial homeostasis and enhanced oxidative stress. Additionally, altered CHCHD8 expression has been observed in certain cancers, such as glioblastoma and breast cancer, suggesting a role in tumor cell survival and metastasis. Recent research highlights its involvement in cellular responses to hypoxia and metabolic stress, positioning CHCHD8 as a potential therapeutic target for diseases linked to mitochondrial dysfunction. However, its precise molecular mechanisms and signaling networks remain incompletely understood, warranting further investigation into its physiological and pathological roles.
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