Stem Cell-Derived Brain Organoids Model Mitochondrial Diseases

Researchers at the University of Bergen have created mini-brains, also known as brain organoids, using cutting-edge stem cell technology. These organoids can replicate disease processes brought on by mitochondrial failure, which might create new treatment options for severe brain conditions like epilepsy.

The brain depends on mitochondria, sometimes referred to as the powerhouses of cells, to provide energy. Mitochondrial dysfunction can result in serious brain disorders that require a lot of energy to function.

A team of researchers at the University of Bergen's Department of Clinical Medicine, under the direction of Kristina Xiao Liang, has used mini-brains to investigate the effects of genetic mutations in mitochondria on brain cells and other cell types.

The mini-brains give us a unique opportunity to understand disease mechanisms at the cellular level and test potential treatments. This is a significant step towards developing new therapies for diseases like severe epilepsy.”

Kristina Xiao Liang, Department of Clinical Medicine, University of Bergen

Better knowledge of other brain disorders like Parkinson's and Alzheimer's can also result from the research. In a realistic yet controlled setting, the mini-brains provide a useful model for investigating intricate disease processes and evaluating therapeutic approaches.

These diseases often involve mitochondrial dysfunction that can be studied in the mini-brains. They allow researchers to study disease progression in real time, test personalized therapies, and identify new drug targets. While they are still under development, they have shown that we can increase our understanding and treatment of these conditions, potentially revolutionizing the field.”

Kristina Xiao Liang, Department of Clinical Medicine, University of Bergen

Source:
Journal reference:

‌Chen, A., et al. (2024) Hallmark Molecular and Pathological Features of POLG Disease are Recapitulated in Cerebral Organoids. Advanced Science. doi.org/10.1002/advs.202307136.

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