Targeting mitochondrial metabolism to restore neuronal maturation in a murine brain organoid model of Allan-Herndon-Dudley syndrome (AHDS)
Benedetta Lucidi, Francesca Ciarpella, Giulia Pedrotti, Chiara Santanatoglia, Eros Rossi, Emanuela Bottani, Ilaria Decimo · FENS Forum 2024
Thyroid hormone (T3) plays a critical role during early brain and overall central nervous system (CNS) development, and it acts as a major regulator of CNS metabolism and mitochondrial activity.Mutations in the T3 specific receptor monocarboxylate transporter 8 (MCT8) lead to hypothyroidism condition as tough in the Allan Herndon Dudley Syndrome (AHDS), which is characterized by neurodevelopmental defects.We set up a brain organoid model resembling AHDS pathological scenario to study the neurodevelopmental defects and to screen new metabolic target to restore neuronal maturation.AHDS organoids
ePoster · Neuroscience