Assembly and misassembly of mitochondrial respiratory chain Complex I

线粒体是我们细胞中的发电站。它们燃烧我们吃的碳燃料，并通过制造用于肌肉收缩和触发神经等功能的ATP来储存能量。线粒体复合物I是一种分子马达，有助于制造ATP。当复合体I没有正确构建时，经常会出现“线粒体疾病”，这会导致儿童早期死亡。在这个项目中，我们将研究复合体I是如何构建的，以及线粒体如何应对组装问题。

Mitochondria are the powerhouses in our cells. They burn the carbon fuels we eat and store the energy by making ATP that is used for functions such as muscle contraction and triggering of nerves. Mitochondrial Complex I is a molecular motor that helps to make ATP. “Mitochondrial disease” is often seen when Complex I is not built properly and this results in early childhood death. In this project we will study how Complex I is built and how the mitochondria responds to assembly problems.