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.
