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Mitochondrial redox imbalance and CoQ10 deficiency in Rett syndrome: Insights from patient-derived fibroblasts.

Archives of biochemistry and biophysics - 1 Dec 2025

Mengarelli Francesco, Cordone Valeria, Pecorelli Alessandra, Benedusi Mascia, Valacchi Giuseppe, Morresi Camilla, Bacchetti Tiziana, Orlando Patrick, Tiano Luca, Silvestri Sonia

Abstract excerpt

Rett syndrome (RTT), a neurodevelopmental disorder primarily affecting females, is characterized by mutations in the MECP2 gene, leading to systemic oxidative stress and mitochondrial dysfunction. This study investigates the role of Coenzyme Q10 (CoQ10), particularly its reduced form ubiquinol, in modulating oxidative stress and mitochondrial function in primary dermal fibroblasts derived from RTT patients with...

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Source

Identifiers

PMID: 41062050
DOI: 10.1016/j.abb.2025.110636

Genes

MECP2
PON2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026.

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