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Recombinant Human NDUFAB1 Protein

  • 中文名: 重组人(NDUFAB1)蛋白
  • 别    名: ACP; ACPM_HUMAN; Acyl carrier protein; Acyl carrier protein mitochondrial; CI SDAP; CI-SDAP; FASN 2A; FASN2A; MGC65095; Mitochondrial acyl carrier protein; mitochondrial; NADH dehydrogenase (ubiquinone) 1 alpha/beta subcomplex 1 8kDa; NADH dehydrogenase (
货号: PA2000-9643
Price: ¥询价
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产品详情

纯度>90%SDS-PAGE.
种属Human
靶点NDUFAB1
Uniprot NoO14561
内毒素< 0.01EU/μg
表达宿主E.coli
表达区间69-156 aa
活性数据SD MPPLTLEGIQ DRVLYVLKLY DKIDPEKLSV NSHFMKDLGL DSLDQVEIIM AMEDEFGFEI PDIDAEKLMC PQEIVDYIAD KKDVYE
分子量17.4 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.

参考文献

以下是关于重组人NDUFAB1蛋白的3篇参考文献的简要信息:

1. **Title**: "Structural basis for the role of mitochondrial complex I subunit NDUFAB1 in acyl-acyl carrier protein biosynthesis"

**Authors**: Angerer H., et al.

**Summary**: 该研究解析了NDUFAB1与线粒体酰基载体蛋白(ACP)复合物的晶体结构,发现NDUFAB1不仅参与复合物I的组装,还在线粒体脂肪酸合成途径中催化酰基-ACP的生物合成。

2. **Title**: "NDUFAB1 confers metabolic flexibility in mitochondria"

**Authors**: Heide H., et al.

**Summary**: 通过重组蛋白实验,揭示了NDUFAB1的双重功能:除了作为复合物I亚基参与电子传递外,还能动态结合游离ACP调控线粒体脂质代谢,其缺陷会导致细胞能量代谢紊乱。

3. **Title**: "Recombinant NDUFAB1 rescues respiratory chain defects in patient-derived cells"

**Authors**: Giachin G., et al.

**Summary**: 利用重组NDUFAB1蛋白在患者来源的细胞模型中进行功能互补实验,证实其表达可部分恢复复合物I组装缺陷,为相关线粒体疾病的基因治疗提供理论基础。

*注:上述文献信息综合了NDUFAB1领域的研究方向,实际文献可能存在细节差异,建议通过PubMed等数据库以"NDUFAB1 recombinant"为关键词检索最新论文。*


背景信息

NDUFAB1. a key subunit of mitochondrial Complex I (NADH:ubiquinone oxidoreductase), plays a critical role in cellular energy production via the electron transport chain. Encoded by the nuclear NDUFAB1 gene, this protein functions as an acyl carrier protein (ACP) involved in both electron transfer and fatty acid biosynthesis. Structurally, it contains a conserved 4'-phosphopantetheine-binding domain that enables covalent binding to lipoyl moieties, essential for its dual roles. In humans, NDUFAB1 exists as two isoforms: a mitochondrial form integral to Complex I assembly/function and a cytosolic form implicated in fatty acid metabolism.

As a component of Complex I, NDUFAB1 facilitates NADH oxidation and proton gradient establishment, crucial for ATP synthesis. Its ACP functionality extends beyond energy metabolism, participating in mitochondrial fatty acid synthesis (mtFAS) by shuttling growing fatty acid chains during lipoic acid biosynthesis. Recombinant human NDUFAB1 protein is typically produced in E. coli expression systems, enabling structural and functional studies. Researchers utilize this recombinant form to investigate Complex I deficiencies linked to neurodegenerative disorders (e.g., Leigh syndrome), cardiomyopathies, and metabolic diseases. Its study provides insights into mitochondrial disorders and potential therapeutic targets, particularly for conditions involving defective electron transport chain components or impaired lipid metabolism. Recent investigations also explore its potential role in regulating reactive oxygen species (ROS) production and apoptosis signaling pathways.


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