Marsy Sophie, Frachon Paule, Dujardin Geneviève, Lombès Anne, Lemaire Claire
CNRS, Centre de Génétique Moléculaire, Avenue de la Terrasse, 91198 Gif-sur-Yvette cedex, France.
FEBS Lett. 2008 Oct 15;582(23-24):3489-93. doi: 10.1016/j.febslet.2008.09.016. Epub 2008 Sep 18.
Pleiotropic effects in the oxidative phosphorylation pathway (OXPHOS) were investigated in yeast respiratory mutants and in cells from patients with OXPHOS genetic alterations. The main differences between yeast and human cells were (1) the site of the primary defect that was associated with pleiotropic effects, yeast complex V and human complex IV, and (2) the nature of the complex targeted by the secondary effect, yeast complex IV and human complex I. The pleiotropic effects did not correlate with the organization of OXPHOS into supercomplexes and their functional consequences appeared to be a slowing down of the respiratory chain in order to avoid either an increase in the membrane potential or the accumulation of reduced intermediary components of the respiratory chain.
在酵母呼吸突变体以及氧化磷酸化（OXPHOS）基因改变患者的细胞中，对氧化磷酸化途径中的多效性效应进行了研究。酵母细胞与人类细胞之间的主要差异在于：（1）与多效性效应相关的主要缺陷位点，酵母中的复合体V和人类中的复合体IV；（2）次要效应所靶向的复合体的性质，酵母中的复合体IV和人类中的复合体I。多效性效应与氧化磷酸化组装成超复合体的过程并无关联，其功能后果似乎是呼吸链的减缓，以避免膜电位升高或呼吸链还原中间组分的积累。
