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在CLPP缺陷组织中翻译忠实性和呼吸缺陷:来自线粒体复合体剖析的机械洞察力

Jana Key1, Suzana Gispert1, Gabriele Koepf1

  • 1Goethe University Frankfurt, University Hospital, Clinic of Neurology, Exp. Neurology, Heinrich Hoffmann Str. 7, 60590 Frankfurt am Main, Germany.

International journal of molecular sciences
|December 23, 2023
PubMed
概括

在3型佩罗氏综合征中,线粒体酶CLPP损失导致蛋白质积聚和线粒体翻译受损,导致神经退行和听力损失.

关键词:
亚精子缺血症 (Azoospermia) 是一种mtLSU 质量控制 质量控制原发性卵巢功能不充分症.这种rRNA伴侣ERAL1是ERAL1.感应神经神经听力损失

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The Supercomplexes in the Crista Membrane01:41

The Supercomplexes in the Crista Membrane

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...