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相关概念视频

Electron Transport Chain: Complex III and IV01:43

Electron Transport Chain: Complex III and IV

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During the electron transport chain, electrons from NADH and FADH2 are first transferred to complexes I and II, respectively. These two complexes then transfer the electrons to ubiquinol, which carries them further to complex III. Complex III passes the electrons across the intermembrane space to Cyt c, which carries them further to complex IV. Complex IV donates electrons to oxygen and reduces it to water. As electrons pass through complexes I, III, and IV, the energy released aids the pumping...
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Electron Transport Chain: Complex I and II01:46

Electron Transport Chain: Complex I and II

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The mitochondrial electron transport chain (ETC) is the main energy generation system in the eukaryotic cells. However, mitochondria also produce cytotoxic reactive oxygen species (ROS) due to the large electron flow during oxidative phosphorylation. While Complex I is one of the primary sources of superoxide radicals, ROS production by Complex II is uncommon and may only be observed in cancer cells with mutated complexes.
ROS generation is regulated and maintained at moderate levels necessary...
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Electron Transport Chains01:28

Electron Transport Chains

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The final stage of cellular respiration is oxidative phosphorylation that consists of two steps: the electron transport chain and chemiosmosis. The electron transport chain is a set of proteins found in the inner mitochondrial membrane in eukaryotic cells. Its primary function is to establish a proton gradient that can be used during chemiosmosis to produce ATP and generate electron carriers, such as NAD+ and FAD, that are used in glycolysis and the citric acid cycle.
The ETC is comprised of...
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The Supercomplexes in the Crista Membrane01:41

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The mitochondrial cristae membrane is the primary site for the oxidative phosphorylation (OXPHOS) process of energy conversion mediated through respiratory complexes I to V. These complexes have been widely studied for decades, and it has been proven that they form supramolecular structures called respiratory supercomplexes (SC). These higher-order complexes may be crucial in maintaining the biochemical structure and improving the physiological activity of the individual complexes while...
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Electron Transport Chain Components01:29

Electron Transport Chain Components

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The electron transport chain (ETC) is a crucial metabolic pathway that facilitates energy conversion in prokaryotic and eukaryotic cells. In eukaryotes, the ETC comprises four membrane-associated protein complexes in the inner mitochondrial membrane. In prokaryotes, the ETC in the plasma membrane can vary in composition, with fewer or different complexes depending on the organism and environmental conditions. These complexes transfer electrons from electron donors, such as NADH and FADH2, to...
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The Electron Transport Chain01:30

The Electron Transport Chain

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The electron transport chain or oxidative phosphorylation is an exothermic process in which free energy released during electron transfer reactions is coupled to ATP synthesis. This process is a significant source of energy in aerobic cells, and therefore inhibitors of the electron transport chain can be detrimental to the cell's metabolic processes.
Inhibitors of the electron transport chain
Rotenone, a widely used pesticide, prevents electron transfer from Fe-S cluster to ubiquinone or Q...
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相关实验视频

Updated: Sep 10, 2025

Author Spotlight: Advancing Techniques and Discoveries in Protein Synthesis and Assembly Through Innovative Mitochondrial Research
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在COXPD8中功能障碍的电子传输链组件

Gisela Beutner1, Heidie L Huyck2, Gail Deutsch3

  • 1Department of Pediatrics-Division Cardiology, University of Rochester Medical Center, Rochester, NY 14642, USA.

Journal of cardiovascular development and disease
|August 27, 2025
PubMed
概括
此摘要是机器生成的。

由AARS2基因突变引起的组合氧化酸化缺陷8型 (COXPD8) 会导致严重的婴儿疾病. 这些突变损害了电子运输链的复杂组合和超级复杂的形成,导致生物能量压力.

关键词:
其他:电子输送链缩性心肌病症线粒体疾病线粒体超级复合体

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Inner Mitochondrial Membrane Sensitivity to Na+ Reveals Partially Segmented Functional CoQ Pools
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科学领域:

  • 线粒体生物学
  • 遗传学
  • 生物化学

背景情况:

  • 组合氧化酸化缺陷8型 (COXPD8) 是一种严重的自身遗传性线粒体疾病.
  • 它是由核编码的线粒体alanil- tRNA合成酶基因 (AARS2) 的突变引起的.
  • 临床特征包括婴儿多性心肌病,肺部缺血,肌肉衰弱和神经问题.

研究的目的:

  • 在患有新型AARS2突变的患者中研究COXPD8的分子机制.
  • 分析这些突变对线粒体呼吸链复合体组合和功能的影响.
  • 描述AARS2突变的生物能量后果.

主要方法:

  • 对AARS2基因突变的基因分析 (c.1738 C>G和c.2872 C>T).
  • 使用LungMAP程序对心脏组织进行分析,以评估电子传输链 (ETC) 复杂组合和超复杂形成.
  • 对ETC成分和三碳酸循环酶的蛋白质表达分析.

主要成果:

  • 这名患者携带了两个AARS2突变, 其中一个突变以前没有被报告.
  • 这些突变破坏了ETC的功能单体复合物I和IV的组合.
  • 在关键TCA循环酶的正常表达的同时,观察到呼吸超复合体的形成减少和某些ETC蛋白的表达改变.

结论:

  • 发现的AARS2突变与ETC复合物I和IV的组装失败有关.
  • 减少呼吸超复合体的形成有助于COXPD8的生物能量压力.
  • 这些发现阐明了COXPD8的分子病理,并突出了AARS2在线粒体功能中的作用.