Bezafibrate improves mitochondrial function in the CNS of a mouse model of mitochondrial encephalopathy

Natalie Noe, Lloye Dillon, Veronika Lellek, Francisca Diaz, Aline Hida, Carlos T Moraes, Tina Wenz

Research output: Contribution to journalArticle

37 Citations (Scopus)

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 languageEnglish
Pages (from-to)417-426
Number of pages10
JournalMitochondrion
Volume13
Issue number5
DOIs
StatePublished - Sep 1 2013

Fingerprint

Bezafibrate
Cytochrome-c Oxidase Deficiency
Mitochondrial Proteins
Gene Deletion
Neuroprotective Agents
Prosencephalon
Neurodegenerative Diseases
Central Nervous System
Adenosine Triphosphate
Mitochondrial encephalopathy
Phenotype

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 journalArticle

Noe, Natalie ; Dillon, Lloye ; Lellek, Veronika ; Diaz, Francisca ; Hida, Aline ; Moraes, Carlos T ; Wenz, Tina. / Bezafibrate improves mitochondrial function in the CNS of a mouse model of mitochondrial encephalopathy. In: Mitochondrion. 2013 ; Vol. 13, No. 5. pp. 417-426.
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