Aberrant mitochondrial bioenergetics in the cerebral cortex of the Fmr1 knockout mouse model of fragile X syndrome
-
Simona D’Antoni
und Maria Vincenza Catania
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.
Acknowledgements
This work was supported by CNR, Oasi Research Institute – IRCCS, Troina and TELETHON foundation (GGP07264). We thank Giuseppina Barrancotto (Oasi Research Institute – IRCCS, Troina) and Barbara De Marzo (IBIOM-CNR, Bari) for technical support.
Conflict of interest statement: There are no conflicts of interest to declare.
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Artikel in diesem Heft
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Artikel in diesem Heft
- Frontmatter
- Reviews
- Aha-type co-chaperones: the alpha or the omega of the Hsp90 ATPase cycle?
- Developments in anticancer vaccination: budding new adjuvants
- The cGMP system: components and function
- Minireview
- Platelets as a ‘natural factory’ for growth factor production that sustains normal (and pathological) cell biology
- Research Articles/Short Communications
- Genes And Nucleic Acids
- Hsa-miR-6165 downregulates insulin-like growth factor-1 receptor (IGF-1R) expression and enhances apoptosis in SW480 cells
- Circular RNA hsa_circ_0001178 facilitates the invasion and metastasis of colorectal cancer through upregulating ZEB1 via sponging multiple miRNAs
- Molecular Medicine
- Aberrant mitochondrial bioenergetics in the cerebral cortex of the Fmr1 knockout mouse model of fragile X syndrome
- Cell Biology and Signaling
- Bone marrow-derived mesenchymal stem cells utilize the notch signaling pathway to induce apoptosis of hepatic stellate cells via NF-κB sensor
- Different signaling and functionality of Rac1 and Rac1b in the progression of lung adenocarcinoma