کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
2068747 | 1078348 | 2013 | 10 صفحه PDF | دانلود رایگان |
![عکس صفحه اول مقاله: Bezafibrate improves mitochondrial function in the CNS of a mouse model of mitochondrial encephalopathy Bezafibrate improves mitochondrial function in the CNS of a mouse model of mitochondrial encephalopathy](/preview/png/2068747.png)
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.
► Bezafibrate induces mitochondrial biogenesis in neuronal cells.
► Bezafibrate has a therapeutic benefit in a mouse model of mitochondrial encephalopathy.
► Bezafibrate attenuate astrogliosis.
Journal: Mitochondrion - Volume 13, Issue 5, September 2013, Pages 417–426