Mitochondrial ATP synthase: cellular power generator, determinant of mitochondrial cristae formation, a site linked to human diseases

来自太阳的能量被光合作用捕获并储存在我们从食物中摄取的高能量化合物中。能量通过我们身体细胞中线粒体的受控燃烧释放出来，并储存在高能量化合物三磷酸腺苷（ATP）中，ATP是生物的燃料。ATP是在我们细胞内的线粒体中产生的。它们是充满了数百万个分子涡轮机的“发电站”，ATP合成酶像人造涡轮机一样旋转，大量生产ATP。我们知道这些分子涡轮机是如何工作的，以及旋转是如何产生的。现在我们想了解哺乳动物酶的活性是如何被调节的，它是如何由19个单独的组成蛋白质组合在一起的，以及去除单个亚基对嵴超微结构的影响。

Energy from the sun is entrapped by photosynthesis and stored in high energy compounds that we consume in food. The energy is released by controlled burning in the mitochondria in the cells of our bodies, and stored in the high energy compound adenosine triphosphate, ATP, the fuel of biology. ATP is made in the mitochondria inside our cells. They are “power stations” full of millions of molecular turbines, the ATP synthases that rotate like man-made turbines and churn out ATP in massive quantities.We understand how these molecular turbines work, and how rotation is generated. Now we want to understand how the activity of the mammalian enzyme is regulated, how it is put together from nineteen individual constituent proteins, and the impact on the ultrastructure of the cristae of removing individual subunits.