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 |
|---|---|
| Pages (from-to) | 417-426 |
| Number of pages | 10 |
| Journal | Mitochondrion |
| Volume | 13 |
| Issue number | 5 |
| DOIs | |
| State | Published - Sep 1 2013 |
Fingerprint
Keywords
- Bezafibrate
- CNS
- Mitochondrial encephalopathy
- PPAR/PGC-1α
ASJC Scopus subject areas
- Cell Biology
- Molecular Biology
- Molecular Medicine
Cite this
Bezafibrate improves mitochondrial function in the CNS of a mouse model of mitochondrial encephalopathy. / Noe, Natalie; Dillon, Lloye; Lellek, Veronika; Diaz, Francisca; Hida, Aline; Moraes, Carlos T; Wenz, Tina.
In: Mitochondrion, Vol. 13, No. 5, 01.09.2013, p. 417-426.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Bezafibrate improves mitochondrial function in the CNS of a mouse model of mitochondrial encephalopathy
AU - Noe, Natalie
AU - Dillon, Lloye
AU - Lellek, Veronika
AU - Diaz, Francisca
AU - Hida, Aline
AU - Moraes, Carlos T
AU - Wenz, Tina
PY - 2013/9/1
Y1 - 2013/9/1
N2 - 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.
AB - 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.
KW - Bezafibrate
KW - CNS
KW - Mitochondrial encephalopathy
KW - PPAR/PGC-1α
UR - http://www.scopus.com/inward/record.url?scp=84882827626&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84882827626&partnerID=8YFLogxK
U2 - 10.1016/j.mito.2012.12.003
DO - 10.1016/j.mito.2012.12.003
M3 - Article
C2 - 23261681
AN - SCOPUS:84882827626
VL - 13
SP - 417
EP - 426
JO - Mitochondrion
JF - Mitochondrion
SN - 1567-7249
IS - 5
ER -