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

Simona D'Antoni, Lidia De Bari, Daniela Valenti, Marina Borro, Carmela Maria Bonaccorso, Maurizio Simmaco, Rosa Anna Vacca, Maria Vincenza Catania

Research output: Contribution to journalArticle

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.

Original languageEnglish
JournalBiological Chemistry
DOIs
Publication statusPublished - Jan 1 2019

Keywords

  • brain cortex mitochondria
  • Fmr1 KO mice
  • glycolytic enzymes
  • mitochondrial glycerol-3-phosphate dehydrogenase
  • mitochondrial respiratory chain
  • oxidative phosphorylation

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Clinical Biochemistry

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