Abstract

Human mitochondria possess one of the most simplified genomes in cellular life. Despite the genome simplicity, the regulatory mechanisms of mitochondrial gene expression remain significant outstanding biological questions. In my group, we investigate the quality control of mitochondrial gene expression, focusing on post-transcriptional and co-translational processes, and how disruptions to these molecular functions manifest into human pathologies. My goal is to understand how these mechanisms are then integrated with organelle membrane dynamics and function across cell types. Our current studies have identified underlying sources of RNA errors in mitochondrial gene expression and the associated ribosome quality control machinery to resolve these aberrations, and how disruptions to these processes manifest across diverse cell types and in animal models of mitochondrial dysfunction. Understanding cell-specific responses to mitochondrial protein synthesis defects will be a key to elucidate the molecular pathogenesis of human mitochondrial disorders.