Yoon Lab

Neurodegenerative and neurodevelopmental diseases affect millions of people around the globe. Scientists have thought many of these diseases may result from disruptions or failures in the mitochondria, a specialized cellular structure often referred to as the “powerhouse of the cell.”

Mitochondria play a key role in metabolism in cells. Metabolism is a series of biochemical reactions essential for all living organisms. It provides energy and the building blocks for healthy cells. As we age, we lose some of these capabilities of mitochondria, and the quality of our cells declines. This has been implicated in many diseases, including neurodegenerative diseases like Alzheimer’s and Parkinson’s and even cancer.

Our long-term goal is to learn how cells maintain mitochondrial structure and metabolism, how failures in that system may contribute to human pathologies and, ultimately, to find treatments for those diseases.

To do this, we use common fruit flies called Drosophila as a disease model. Because fruit flies share approximately 75 percent of a human’s disease genes, they are near-perfect tools for studying conditions that afflict people. We “humanize” these flies by using state-of-the-art gene editing tools. By studying how their mitochondrial dysfunction contributes to defects in flies, we can get hints into the root causes for human diseases.

The Lab also focuses on identifying novel and previously unknown human diseases using the newest technologies in Drosophila. In collaboration with human geneticists, I discovered mitochondrial genes whose mutations cause neuronal and metabolic dysfunction in both flies and humans. This work shows that fruit flies serve as an excellent model system for discovering novel disease genes from human patient data. Using these tiny flies, we can continue to identify new human diseases, perform disease modeling in flies, and understand the mechanisms of their pathogenesis.

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