Complex II proteostasis in mammalian mitochondria.

线粒体在真核细胞的能量传递和其他代谢过程中起着至关重要的作用。绝大多数执行这些功能的蛋白质都是核编码的，并在它们输入后在线粒体中折叠和组装。本项目旨在确定呼吸复合体II的组装过程中蛋白质复合体生物发生和质量控制的动态和竞争过程之间的相互作用。该项目的目的是确定组装途径的步骤和分子组成，揭示唯一已知的黄素化因子的分子机制，并确定蛋白酶对该途径的影响。该项目意义重大，因为线粒体蛋白质稳态失衡会导致人类疾病。

Mitochondria are critical for energy transduction and other metabolic processes in eukaryotic cells. The vast majority of proteins that perform these functions are nuclear-encoded and fold and assemble in the mitochondrion after their import. This project aims to determine the interplay between the dynamic and competing processes of protein complex biogenesis and quality control in the assembly of respiratory complex II. The project aims to determine the steps and molecular components of the assembly pathway, uncover the molecular mechanism of the only known flavinylation factor and to establish the impact of proteases on this pathway. The project is significant because an imbalance in mitochondrial protein homeostasis leads to diseases in humans.