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Aberrant mitochondrial bioenergetics in the cerebral cortex of the Fmr1 knockout mouse model of fragile X syndrome

Authors
  • D’Antoni, Simona1,
  • de Bari, Lidia2,
  • Valenti, Daniela2,
  • Borro, Marina3
  • Bonaccorso, Carmela Maria4
  • Simmaco, Maurizio3
  • Vacca, Rosa Anna2
  • Catania, Maria Vincenza1, 4
  • 1 National Research Council (CNR), Via Paolo Gaifami 18 , (Italy)
  • 2 Institute of Biomembranes, Bioenergetics and Molecular Biotechnologies (IBIOM), CNR, Via Giovanni Amendola 165/A , (Italy)
  • 3 Sapienza University of Rome, Via di Grottarossa 1035 , (Italy)
  • 4 Oasi Research Institute – IRCCS, Via Conte Ruggero 73 , (Italy)
Type
Published Article
Journal
Biological Chemistry
Publisher
Walter de Gruyter GmbH
Publication Date
Nov 08, 2019
Volume
401
Issue
4
Pages
497–503
Identifiers
DOI: 10.1515/hsz-2019-0221
Source
De Gruyter
Keywords
License
Yellow

Abstract

Impaired energy metabolism may play a role in the pathogenesis of neurodevelopmental disorders including fragile X syndrome (FXS). We checked brain energy status and some aspects of cell bioenergetics, namely the activity of key glycolytic enzymes, glycerol-3-phosphate shuttle and mitochondrial respiratory chain (MRC) complexes, in the cerebral cortex of the Fmr1 knockout (KO) mouse model of FXS. We found that, despite a hyperactivation of MRC complexes, adenosine triphosphate (ATP) production via mitochondrial oxidative phosphorylation (OXPHOS) is compromised, resulting in brain energy impairment in juvenile and late-adult Fmr1 KO mice. Thus, an altered mitochondrial energy metabolism may contribute to neurological impairment in FXS.

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