Human brain organoids with mature neurons in green (pos. for the marker MAP2) (IMAGE)

Heinrich-Heine University Duesseldorf

Human brain organoids with mature neurons in green (pos. for the marker MAP2)

Caption

In brain organoids from Leigh syndrome patients, neurons do not mature properly. This is due to an impaired activation of mitochondrial metabolism in cells that generate neurons which are called neural precursors. The authors demonstrate that helping the activation of mitochondrial metabolism in neural precursors can revert the defects. They achieved this using two strategies that may be applicable in the clinics: 1) gene correction with CRISPR/Cas9 or viral-based delivery of the healthy gene, 2) increasing the expression of the metabolic regulator PGC1-alpha using the FDA-approved drug Bezafibrate.

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Copyright: Dr. Agnieszka Rybak-Wolf

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Must not be published without permission of Dr. Agnieszka Rybak-Wolf. Please contact Prof. Prigione for contact.

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