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Mini-brains reveal how mitochondrial mutations affect brain cells

Breakthrough in stem cell research: Mini-brains provide insight into mitochondrial diseases
The mini-brain samples are safely stored in the laboratory. They are so tiny that they need to be examined in the microscope. Credit: University of Bergen

Researchers at the University of Bergen have used advanced stem cell technology to develop mini-brains, also called brain organoids, that can mimic disease processes caused by mitochondrial failure. This could open new avenues for treating serious brain diseases such as epilepsy.

Mitochondria are crucial for supplying the brain with energy. When mitochondrial function fails, this can lead to serious disorders in the brain, which require a lot of energy to function.

Using mini-brains, a research team led by Kristina Xiao Liang at the Department of Clinical Medicine, University of Bergen, has been able to study how genetic mutations in mitochondria affect brain cells and other cell types. Their work is published in the journal Advanced Science.

“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,” says Liang.

  • Breakthrough in stem cell research: Mini-brains provide insight into mitochondrial diseases
    Kristina Xiao Liang is Group Leader in Mitochondrial Stem Cell Research Group at the University of Bergen. Credit: University of Bergen
  • Breakthrough in stem cell research: Mini-brains provide insight into mitochondrial diseases
    To build the mini-brains, the scientists coax clusters of stem cells into becoming neurons and other brain cells. This offers a unique tool for studying human brain development and the effects of disorders. Credit: University of Bergen

In addition, the research can contribute to a better understanding of other brain diseases such as Alzheimer’s and Parkinson’s disease. The mini-brains offer a valuable model for exploring complex disease processes and testing treatment strategies in a realistic but controlled environment.

“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,” says Liang.

More information:
Anbin Chen et al, Hallmark Molecular and Pathological Features of POLG Disease are Recapitulated in Cerebral Organoids, Advanced Science (2024). DOI: 10.1002/advs.202307136

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Mini-brains reveal how mitochondrial mutations affect brain cells (2024, December 13)
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