Using Drosophila to study and identify new therapies for mitochondrial disease

Mitochondrial diseases are a common group of metabolic disorders that affect tissues that require high levels of energy, such as the brain and muscle. Patients with mitochondrial disease can suffer from a range of debilitating symptoms that compromise quality and length of life. Critically, there are no curative treatments available for people with these diseases. We need to know more about what happens when mitochondrial function becomes compromised and how these events link to mitochondrial disease so that we can accelerate the development of new treatments. Evidence from previous studies suggests that defective mitochondrial function results in adaptations that may cause mitochondrial disease. Targeting these adaptations may be a route to finding effective therapies.

The common fruit fly has been used successfully to model a number of human diseases, including mitochondrial disorders. I will use the fly as a model to study the adaptations that occur when mitochondrial function is impaired in the whole fly and in tissues such as the brain and muscle. I will also explore whether reinstatement of normal mitochondrial function can reverse established mitochondrial disease. Finally, I will establish a screening method using fly models to identify therapies that could alleviate mitochondrial disease.