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Recombinant Human NDUFB8 Protein

  • 中文名: 重组人(NDUFB8)蛋白
  • 别    名: NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8. mitochondrial; Complex I-ASHI; CI-ASHI; NADH-ubiquinone oxidoreductase ASHI subunit
货号: PA2000-9650
Price: ¥询价
数量:
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产品详情

纯度>90%SDS-PAGE.
种属Human
靶点NDUFB8
Uniprot NoO95169
内毒素< 0.01EU/μg
表达宿主E.coli
表达区间1-186  aa
活性数据MAVARAGVLGVQWLQRASRNVMPLGARTASHMTKDMFPGPYPRTPEERAAAAKKYNMRVEDYEPYPDDGMGYGDYPKLPDRSQHERDPWYSWDQPGLRLNWGEPMHWHLDMYNRNRVDTSPTPVSWHVMCMQLFGFLAFMIFMCWVGDVYPVYQPVGPKQYPYNNLYLERGGDPSKEPERVVHYEI
分子量46.2 kDa
蛋白标签GST-tag at N-terminal
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.

参考文献

以下是关于重组人NDUFB8蛋白的3篇代表性文献摘要(作者和文献名称部分为模拟示例,实际引用时需核对具体文献):

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1. **文献名称**:*"Structural insights into the role of NDUFB8 in mitochondrial complex I assembly"*

**作者**:Smith J et al.

**摘要**:本研究通过冷冻电镜技术解析了人源NDUFB8蛋白在复合物I中的结构,揭示其作为核心亚基在复合物组装及电子传递链稳定性中的作用,并发现其突变可能导致复合物I功能障碍。

2. **文献名称**:*"Recombinant NDUFB8 expression rescues mitochondrial dysfunction in cellular models of Leigh syndrome"*

**作者**:Li Y et al.

**摘要**:研究利用HEK293细胞表达重组人NDUFB8蛋白,证明其在体外可恢复因NDUFB8缺失导致的线粒体膜电位下降和ATP合成缺陷,提示其作为线粒体脑肌病潜在治疗靶点。

3. **文献名称**:*"NDUFB8 mutations impair respiratory chain activity and induce fragmented mitochondria in Parkinson’s disease models"*

**作者**:Garcia-Ruiz C et al.

**摘要**:通过CRISPR编辑技术构建NDUFB8敲除细胞模型,发现其缺失导致线粒体碎片化及ROS过度积累,进一步在帕金森病小鼠模型中验证重组NDUFB8蛋白的神经保护作用。

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**注**:以上文献信息为示例,实际研究中请通过PubMed或Web of Science检索真实文献(可尝试关键词:NDUFB8 + recombinant/complex I/mitochondrial disease)。如需具体文献协助,请补充研究背景。


背景信息

Recombinant human NDUFB8 protein is a key subunit of mitochondrial Complex I (NADH:ubiquinone oxidoreductase), the largest enzyme in the electron transport chain (ETC) responsible for oxidative phosphorylation. Complex I facilitates the transfer of electrons from NADH to ubiquinone, coupled with proton pumping across the mitochondrial inner membrane to generate ATP. NDUFB8. encoded by the nuclear DNA, is an iron-sulfur (Fe-S) cluster-binding protein critical for the structural integrity and catalytic activity of Complex I. It resides in the membrane arm of the enzyme and plays a role in stabilizing interactions between core subunits.

Dysfunction of NDUFB8 is linked to mitochondrial disorders, neurodegenerative diseases, and cancer due to impaired energy metabolism. Recombinant NDUFB8 is produced using heterologous expression systems (e.g., *E. coli* or mammalian cells) for functional studies, antibody production, and disease modeling. Its molecular weight is approximately 21 kDa, and post-translational modifications may be retained depending on the expression system. Research applications include investigating Complex I assembly defects, screening for metabolic disease biomarkers, and exploring therapeutic targets. The recombinant protein serves as a vital tool in understanding mitochondrial biology and pathologies associated with ETC dysfunction.


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