Mitochondria are vital parts of our cells that play a major role in energy conversion. Thereby, they apply oxygen from the air we breathe and metabolites derived from our food to produce ATP, the main form of energy used in our cells. This makes mitochondria the foundation of all oxygen-dependent life on Earth. Interestingly, mitochondria originated as separate organisms and were incorporated into our cells during evolution. Thus, they contain their own DNA, which codes for proteins that are essential for the energy conversion process.
To ensure that proteins function properly, they are monitored by complex systems in a process known as protein quality control (PQC). PQC ensures accurate protein production, proper transport and placement within the cell, correct folding into functional proteins, and, if necessary, breakdown of damaged or surplus proteins. This is particularly important for mitochondria, as their oxidative environment can damage proteins and disrupt cellular functions. Indeed, the accumulation of such damage is associated with ageing and can result in age-related diseases, including cancer and neurodegenerative disorders like Parkinson’s disease. However, many aspects of mitochondrial PQC are still unknown, especially PQC of proteins encoded by mitochondrial DNA remains enigmatic.
Our research aims to uncover the principles of PQC in mitochondria. We focus on proteins encoded by mitochondrial DNA and use cutting-edge biochemical methods for spatiotemporal analysis of the underlying mechanisms. Using the yeast Saccharomyces cerevisiae and human cells, we study these processes in young vs. aged cells to understand how mitochondrial PQC works in healthy cells, how these processes change with age, and identify points of onset for age-related diseases. Our goal is to expand the knowledge on mitochondrial PQC and pave the way for potential future treatments against human diseases.
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