Article
Mitochondrial Dysfunction Causes Cell Death in Patients Affected by Fragile-X-Associated Disorders.
International journal of molecular sciences - 18 Mar 2024
Grandi Martina, Galber Chiara, Gatto Cristina, Nobile Veronica, Pucci Cecilia, Schaldemose Nielsen Ida, Boldrin Francesco, Neri Giovanni, Chiurazzi Pietro, Solaini Giancarlo, Baracca Alessandra, Giorgio Valentina, Tabolacci Elisabetta
Abstract excerpt
Mitochondria are involved in multiple aspects of neurodevelopmental processes and play a major role in the pathogenetic mechanisms leading to neuro-degenerative diseases. Fragile-X-related disorders (FXDs) are genetic conditions that occur due to the dynamic expansion of CGG repeats of the FMR1 gene encoding for the RNA-binding protein FMRP, particularly expressed in the brain. This gene expansion can lead to...
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