Biosci Rep. 2013 Sep 17;33(5):e00067. doi: 10.1042/BSR20130078.
IF1 is an endogenous inhibitor protein of mitochondrial ATP synthase. It is evolutionarily conserved throughout all eukaryotes and it has been proposed to play crucial roles in prevention of the wasteful reverse reaction of ATP synthase, in the metabolic shift from oxidative phosphorylation to glycolysis, in the suppression of ROS (reactive oxygen species) generation, in mitochondria morphology and in haem biosynthesis in mitochondria, which leads to anaemia. Here, we report the phenotype of a mouse strain in which IF1 gene was destroyed. Unexpectedly, individuals of this IF1-KO (knockout) mouse strain grew and bred without defect. The general behaviours, blood test results and responses to starvation of the IF1-KO mice were apparently normal. There were no abnormalities in the tissue anatomy or the autophagy. Mitochondria of the IF1-KO mice were normal in morphology, in the content of ATP synthase molecules and in ATP synthesis activity. Thus, IF1 is not an essential protein for mice despite its ubiquitous presence in eukaryotes.
IF1 是一种线粒体 ATP 合酶的内源性抑制蛋白。它在所有真核生物中都具有进化保守性，据推测它在防止 ATP 合酶的浪费性逆反应、从氧化磷酸化到糖酵解的代谢转变、抑制 ROS（活性氧）生成、线粒体形态以及在线粒体中促进血红素生物合成以导致贫血等方面发挥着关键作用。在这里，我们报告了一种 IF1 基因被破坏的小鼠品系的表型。出乎意料的是，IF1-KO（敲除）小鼠品系的个体生长和繁殖没有缺陷。IF1-KO 小鼠的一般行为、血液测试结果和对饥饿的反应明显正常。组织解剖或自噬没有异常。IF1-KO 小鼠的线粒体在形态、ATP 合酶分子含量和 ATP 合成活性方面均正常。因此，尽管 IF1 在真核生物中普遍存在，但它不是小鼠的必需蛋白。
