Abstract
Mitochondrial dysfunction frequently affects the central nervous system. Here, we investigated the effect of bezafibrate treatment on neuronal mitochondrial function and its impact on the progression of a mitochondrial encephalopathy. We used a murine model with a forebrain-specific cytochrome c oxidase deficiency caused by conditional deletion of the COX10 gene. In this mouse model, bezafibrate-administration improved the phenotype of the mice associated with an increase in mitochondrial proteins and mitochondrial ATP generating capacity. Bezafibrate-treatment also attenuated astrogliosis and decreased the level of inflammatory markers in the affected tissues. Overall, bezafibrate had a neuroprotective effect in this mouse model of mitochondrial encephalopathy. These findings imply that bezafibrate might be a promising therapeutic agent for the treatment of neurodegenerative disease associated with mitochondrial dysfunction.
Original language | English (US) |
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Pages (from-to) | 417-426 |
Number of pages | 10 |
Journal | Mitochondrion |
Volume | 13 |
Issue number | 5 |
DOIs | |
State | Published - Sep 2013 |
Keywords
- Bezafibrate
- CNS
- Mitochondrial encephalopathy
- PPAR/PGC-1α
ASJC Scopus subject areas
- Cell Biology
- Molecular Biology
- Molecular Medicine