التفاصيل البيبلوغرافية
| العنوان: |
Aberrant mitochondrial bioenergetics in the cerebral cortex of the Fmr1 knockout mouse model of fragile X syndrome. |
| المؤلفون: |
D'Antoni, Simona, de Bari, Lidia, Valenti, Daniela, Borro, Marina, Bonaccorso, Carmela Maria, Simmaco, Maurizio, Vacca, Rosa Anna, Catania, Maria Vincenza |
| المصدر: |
Biological Chemistry; Apr2020, Vol. 401 Issue 4, p497-503, 7p, 1 Chart, 1 Graph |
| مصطلحات موضوعية: |
FRAGILE X syndrome, KNOCKOUT mice, BIOENERGETICS, OXIDATIVE phosphorylation, ENERGY metabolism, PATHOLOGY |
| مستخلص: |
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. [ABSTRACT FROM AUTHOR] |
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| قاعدة البيانات: |
Complementary Index |
