Mitochondria, Metals and Oxidative Stress Laboratory, Institut Jacques Monod, CNRS-Université Paris-Diderot, Sorbonne Paris Cité, 15 rue Hélène Brion, 75205 Paris cedex 13, France.
Biochem Biophys Res Commun. 2012 Feb 10;418(2):336-41. doi: 10.1016/j.bbrc.2012.01.022. Epub 2012 Jan 17.
Friedreich ataxia (FA) is the most common recessive neurodegenerative disease. It is caused by deficiency in mitochondrial frataxin, which participates in iron-sulfur cluster assembly. Yeast cells lacking frataxin (Δyfh1 mutant) showed an increased proportion of fragmented mitochondria compared to wild-type. In addition, oxidative stress induced complete fragmentation of mitochondria in Δyfh1 cells. Genetically controlled inhibition of mitochondrial fission in these cells led to increased resistance to oxidative stress. Here we present evidence that in yeast frataxin-deficiency interferes with mitochondrial dynamics, which might therefore be relevant for the pathophysiology of FA.
弗里德赖希共济失调（FA）是最常见的隐性神经退行性疾病。它是由线粒体 frataxin 缺乏引起的，frataxin 参与铁硫簇组装。与野生型相比，缺乏 frataxin 的酵母细胞（Δyfh1 突变体）显示出更多的线粒体片段化比例。此外，氧化应激诱导 Δyfh1 细胞中线粒体完全碎片化。在这些细胞中，遗传控制的线粒体分裂抑制导致对氧化应激的抗性增加。在这里，我们提供的证据表明，在酵母中，frataxin 缺乏会干扰线粒体动力学，因此这可能与 FA 的病理生理学有关。
