Molecular mechanisms of sensing and repairing dysfunctional mitochondria

Mitochondria are factories in our cells that produce energy and building blocks. Constant delivery of proteins, the factory “workers”, to mitochondria from other parts of the cell is important for proper function of these factories. Defects in delivery occurs in many diseases, including diseases involving nerve cell death (neurodegenerative) like Alzheimer’s. It is thus extremely important and timely to gain more knowledge on how cell health is maintained when protein delivery into mitochondria is damaged.

I discovered a new mechanism, the mitochondrial compromised protein import response (mitoCPR), which protects mitochondria and cells when protein delivery is damaged. I showed that such damage leads to proteins getting stuck and clogging entry sites into mitochondria. My research aims to gain a deeper understanding of how the mitoCPR unclogs mitochondria entry sites and helps them recover under disease and physiological conditions. Using molecular biology and advanced technologies such as gene editing, proteomics, and microscopy, my lab will reveal how the cell keeps mitochondria healthy. This research may uncover new treatment strategies for neurodegenerative and other diseases, caused by improper mitochondrial function.