Journal
BIOLOGICAL CHEMISTRY
Volume 401, Issue 4, Pages 497-503Publisher
WALTER DE GRUYTER GMBH
DOI: 10.1515/hsz-2019-0221
Keywords
brain cortex mitochondria; Fmr1 KO mice; glycolytic enzymes; mitochondrial glycerol-3-phosphate dehydrogenase; mitochondrial respiratory chain; oxidative phosphorylation
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Funding
- CNR
- Oasi Research Institute - IRCCS, Troina
- TELETHON foundation [GGP07264]
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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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