Fighting brain damage by rethinking mitochondria

Albert Quintana

Universitat Autònoma de Barcelona (UAB), Spain

Mitochondrial diseases are rare but devastating conditions that affect around one in every 5,000 children. These diseases damage the body’s energy-producing systems, especially in the brain, leading to progressive and often fatal outcomes. Despite their severity, there are currently no effective treatment, and one of the biggest mysteries is why certain brain cells are more vulnerable than others.

This project explores if damaged mitochondria in brain cells may trigger a false alarm, making the cells believe they are under viral attack. This mistaken response would cause lipids to build up inside the cells, which may lead to further damage and eventually cell death. Using a mouse model that closely mimics the human disease, researchers have already found signs of this faulty immune response and lipids accumulation in the brain. Now, they will use advanced tools to study how this process unfolds in real time. They will examine how lipid droplets form and behave in affected brain cells, and how this relates to the immune system’s response. They will also test whether blocking this response or changing how cells handle fat accummulation can protect the brain and improve symptoms.

The findings could lead to new treatments not only for mitochondrial diseases but also for other conditions related to neurodegenerative disorders where mitochondria are involved.

Lipid droplets as immunometabolic mediators in mitochondrial disease encephalopathy

€499,970.00