TY - JOUR
T1 - Aberrant mitochondrial bioenergetics in the cerebral cortex of the Fmr1 knockout mouse model of fragile X syndrome
AU - D'Antoni, Simona
AU - De Bari, Lidia
AU - Valenti, Daniela
AU - Borro, Marina
AU - Bonaccorso, Carmela Maria
AU - Simmaco, Maurizio
AU - Vacca, Rosa Anna
AU - Catania, Maria Vincenza
PY - 2019/1/1
Y1 - 2019/1/1
N2 - 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.
AB - 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.
KW - brain cortex mitochondria
KW - Fmr1 KO mice
KW - glycolytic enzymes
KW - mitochondrial glycerol-3-phosphate dehydrogenase
KW - mitochondrial respiratory chain
KW - oxidative phosphorylation
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U2 - 10.1515/hsz-2019-0221
DO - 10.1515/hsz-2019-0221
M3 - Article
C2 - 31702995
AN - SCOPUS:85074941490
JO - Biological Chemistry
JF - Biological Chemistry
SN - 1431-6730
ER -