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

Mitochondrion

Volumn 13, Issue 5, 2013, Pages 417-426

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

^a UNIVERSITY OF COLOGNE   (Germany)

^b UNIVERSITY OF MIAMI MILLER SCHOOL OF MEDICINE   (United States)

Author keywords

Bezafibrate; CNS; Mitochondrial encephalopathy; PPAR PGC 1

Indexed keywords

ADENOSINE TRIPHOSPHATE; BEZAFIBRATE; CITRATE SYNTHASE; CYTOCHROME C OXIDASE; GLIAL FIBRILLARY ACIDIC PROTEIN; HEME OXYGENASE 1; INTERLEUKIN 6; MITOCHONDRIAL PROTEIN; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR GAMMA COACTIVATOR 1ALPHA; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE; SUPEROXIDE DISMUTASE; TUBULIN; TUMOR NECROSIS FACTOR ALPHA;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ANTIINFLAMMATORY ACTIVITY; ANTIOXIDANT ACTIVITY; ARTICLE; ASTROCYTOSIS; BRAIN MITOCHONDRION; CONTROLLED STUDY; COX10 GENE; DISEASE MODEL; ENZYME ACTIVITY; ENZYME DEFICIENCY; FOREBRAIN; GENE; GENE DELETION; HUMAN; HUMAN CELL; MITOCHONDRIAL ENCEPHALOPATHY; MOUSE; NEUROPROTECTION; NONHUMAN; OXIDATIVE PHOSPHORYLATION; PHENOTYPE; PRIORITY JOURNAL; TREATMENT OUTCOME;

MURINAE; MUS;

BEZAFIBRATE; CNS; MITOCHONDRIAL ENCEPHALOPATHY; PPAR/PGC-1Α;

ADENOSINE TRIPHOSPHATE; ALKYL AND ARYL TRANSFERASES; ANIMALS; ASTROCYTES; BEZAFIBRATE; CELL PROLIFERATION; DISEASE MODELS, ANIMAL; HYPOLIPIDEMIC AGENTS; MEMBRANE PROTEINS; MICE; MITOCHONDRIA; MITOCHONDRIAL ENCEPHALOMYOPATHIES; MITOCHONDRIAL PROTEINS; NEUROPROTECTIVE AGENTS; TREATMENT OUTCOME;

EID: 84882827626     PISSN: 15677249     EISSN: 18728278     Source Type: Journal    
DOI: 10.1016/j.mito.2012.12.003     Document Type: Article

References (41)

1. Ates O., Cayli S., Altinoz E., Gurses I., Yucel N., Sener M., Kocak A., Yologlu S. Neuroprotection by resveratrol against traumatic brain injury in rats. Mol. Cell. Biochem. 2007, 294:137-144.
2. Beal M.F. Mitochondria and neurodegeneration. Novartis Found. Symp. 2007, 287:183-192. (discussion 192-186).
3. Besson V.C., Chen X.R., Plotkine M., Marchand-Verrecchia C. Fenofibrate, a peroxisome proliferator-activated receptor alpha agonist, exerts neuroprotective effects in traumatic brain injury. Neurosci. Lett. 2005, 388:7-12.
4. Canto C., Auwerx J. PGC-1alpha, SIRT1 and AMPK, an energy sensing network that controls energy expenditure. Curr. Opin. Lipidol. 2009, 20:98-105.
5. Deplanque D., Gele P., Petrault O., Six I., Furman C., Bouly M., Nion S., Dupuis B., Leys D., Fruchart J.C., Cecchelli R., Staels B., Duriez P., Bordet R. Peroxisome proliferator-activated receptor-alpha activation as a mechanism of preventive neuroprotection induced by chronic fenofibrate treatment. J. Neurosci. 2003, 23:6264-6271.
6. Diaz F., Thomas C.K., Garcia S., Hernandez D., Moraes C.T. Mice lacking COX10 in skeletal muscle recapitulate the phenotype of progressive mitochondrial myopathies associated with cytochrome c oxidase deficiency. Hum. Mol. Genet. 2005, 14:2737-2748.
7. Diaz F., Garcia S., Padgett K.R., Moraes C.T. A defect in the mitochondrial complex III, but not complex IV, triggers early ROS-dependent damage in defined brain regions. Hum. Mol. Genet. 2012, 21:5066-5077.
8. Dillon L.M., Hida A., Garcia S., Prolla T.A., Moraes C.T. Long-term bezafibrate treatment improves skin and spleen phenotypes of the mtDNA mutator mouse. PLoS One 2012, 7:e44335.
9. Dillon L.M., Williams S.L., Hida A., Peacock J.D., Prolla T.A., Lincoln J., Moraes C.T. Increased mitochondrial biogenesis in muscle improves aging phenotypes in the mtDNA mutator mouse. Hum. Mol. Genet. 2012, 21:2288-2297.
10. DiMauro S., Schon E.A. Mitochondrial disorders in the nervous system. Annu. Rev. Neurosci. 2008, 31:91-123.
11. Dimauro S., Rustin P. A critical approach to the therapy of mitochondrial respiratory chain and oxidative phosphorylation diseases. Biochim. Biophys. Acta 1792 2009, (12):1159-1167. 10.1016/j.bbadis.2008.10.015.
12. Djouadi F., Bastin J. Species differences in the effects of bezafibrate as a potential treatment of mitochondrial disorders. Cell Metab. 2011, 14:715-716. (author reply 717).
13. Dumont M., Stack C., Elipenahli C., Jainuddin S., Gerges M., Starkova N., Calingasan N.Y., Yang L., Tampellini D., Starkov A.A., Chan R.B., Di Paolo G., Pujol A., Beal M.F. Bezafibrate administration improves behavioral deficits and tau pathology in P301S mice. Hum. Mol. Genet. 2012, 21:5091-5105.
14. Feinstein D.L. Therapeutic potential of peroxisome proliferator-activated receptor agonists for neurological disease. Diabetes Technol. Ther. 2003, 5:67-73.
15. Fukui H., Diaz F., Garcia S., Moraes C.T. Cytochrome c oxidase deficiency in neurons decreases both oxidative stress and amyloid formation in a mouse model of Alzheimer's disease. Proc. Natl. Acad. Sci. U. S. A. 2007, 104:14163-14168.
16. Gonzalez-Scarano F., Baltuch G. Microglia as mediators of inflammatory and degenerative diseases. Annu. Rev. Neurosci. 1999, 22:219-240.
17. Izawa Y., Takahashi S., Suzuki N. Pioglitazone enhances pyruvate and lactate oxidation in cultured neurons but not in cultured astrocytes. Brain Res. 2009, 1305:64-73. 10.1016/j.brainres.2009.09.098.
18. Johri A., Calingasan N.Y., Hennessey T.M., Sharma A., Yang L., Wille E., Chandra A., Beal M.F. Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's disease. Hum. Mol. Genet. 2012, 21:1124-1137.
19. Kapadia R., Yi J.H., Vemuganti R. Mechanisms of anti-inflammatory and neuroprotective actions of PPAR-gamma agonists. Front. Biosci. 2008, 13:1813-1826.
20. Kreisler A., Gele P., Wiart J.F., Lhermitte M., Destee A., Bordet R. Lipid-lowering drugs in the MPTP mouse model of Parkinson's disease: fenofibrate has a neuroprotective effect, whereas bezafibrate and HMG-CoA reductase inhibitors do not. Brain Res. 2007, 1135:77-84.
21. Lin J., Wu H., Tarr P.T., Zhang C.Y., Wu Z., Boss O., Michael L.F., Puigserver P., Isotani E., Olson E.N., Lowell B.B., Bassel-Duby R., Spiegelman B.M. Transcriptional co-activator PGC-1 alpha drives the formation of slow-twitch muscle fibres. Nature 2002, 418:797-801.
22. Luo Y., Yin W., Signore A.P., Zhang F., Hong Z., Wang S., Graham S.H., Chen J. Neuroprotection against focal ischemic brain injury by the peroxisome proliferator-activated receptor-gamma agonist rosiglitazone. J. Neurochem. 2006, 97:435-448.
23. Matsui H., Okumura K., Kawakami K., Hibino M., Toki Y., Ito T. Improved insulin sensitivity by bezafibrate in rats: relationship to fatty acid composition of skeletal-muscle triglycerides. Diabetes 1997, 46:348-353.
24. McGill J.K., Beal M.F. PGC-1alpha, a new therapeutic target in Huntington's disease?. Cell 2006, 127:465-468.
25. Miglio G., Rosa A.C., Rattazzi L., Collino M., Lombardi G., Fantozzi R. PPARgamma stimulation promotes mitochondrial biogenesis and prevents glucose deprivation-induced neuronal cell loss. Neurochem. Int. 2009, 55:496-504.
26. Minghetti L., Levi G. Microglia as effector cells in brain damage and repair: focus on prostanoids and nitric oxide. Prog. Neurobiol. 1998, 54:99-125.
27. Mudo G., Makela J., Liberto V.D., Tselykh T.V., Olivieri M., Piepponen P., Eriksson O., Malkia A., Bonomo A., Kairisalo M., Aguirre J.A., Korhonen L., Belluardo N., Lindholm D. Transgenic expression and activation of PGC-1alpha protect dopaminergic neurons in the MPTP mouse model of Parkinson's disease. Cell Mol. Life Sci. 2012, 69:1153-1165.
28. Mysiorek C., Culot M., Dehouck L., Derudas B., Staels B., Bordet R., Cecchelli R., Fenart L., Berezowski V. Peroxisome-proliferator-activated receptor-alpha activation protects brain capillary endothelial cells from oxygen-glucose deprivation-induced hyperpermeability in the blood-brain barrier. Curr. Neurovasc. Res. 2009, 6:181-193.
29. O'Rourke F., Dean N., Akhtar N., Shuaib A. Current and future concepts in stroke prevention. CMAJ 2004, 170:1123-1133.
30. Pallas M., Casadesus G., Smith M.A., Coto-Montes A., Pelegri C., Vilaplana J., Camins A. Resveratrol and neurodegenerative diseases: activation of SIRT1 as the potential pathway towards neuroprotection. Curr. Neurovasc. Res. 2009, 6:70-81.
31. Ramanan S., Kooshki M., Zhao W., Hsu F.C., Riddle D.R., Robbins M.E. The PPARalpha agonist fenofibrate preserves hippocampal neurogenesis and inhibits microglial activation after whole-brain irradiation. Int. J. Radiat. Oncol. Biol. Phys. 2009, 75:870-877.
32. Ryan M.J., Didion S.P., Mathur S., Faraci F.M., Sigmund C.D. PPAR(gamma) agonist rosiglitazone improves vascular function and lowers blood pressure in hypertensive transgenic mice. Hypertension 2004, 43:661-666.
33. Scarpulla R.C. Transcriptional paradigms in mammalian mitochondrial biogenesis and function. Physiol. Rev. 2008, 88:611-638.
34. St-Pierre J., Drori S., Uldry M., Silvaggi J.M., Rhee J., Jager S., Handschin C., Zheng K., Lin J., Yang W., Simon D.K., Bachoo R., Spiegelman B.M. Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators. Cell 2006, 127:397-408.
35. Tenenbaum A., Motro M., Fisman E.Z. Dual and pan-peroxisome proliferator-activated receptors (PPAR) co-agonism: the bezafibrate lessons. Cardiovasc. Diabetol. 2005, 4:14.
36. Viscomi C., Bottani E., Civiletto G., Cerutti R., Moggio M., Fagiolari G., Schon E.A., Lamperti C., Zeviani M. In vivo correction of COX deficiency by activation of the AMPK/PGC-1alpha axis. Cell Metab. 2011, 14:80-90.
37. Wareski P., Vaarmann A., Choubey V., Safiulina D., Liiv J., Kuum M., Kaasik A. PGC-1{alpha} and PGC-1{beta} regulate mitochondrial density in neurons. J. Biol. Chem. 2009, 284:21379-21385.
38. Wenz T., Diaz F., Spiegelman B.M., Moraes C.T. Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype. Cell Metab. 2008, 8:249-256.
39. Yatsuga S., Suomalainen A. Effect of bezafibrate treatment on late-onset mitochondrial myopathy in mice. Hum. Mol. Genet. 2012, 21:526-535.
40. Yi J.H., Park S.W., Brooks N., Lang B.T., Vemuganti R. PPARgamma agonist rosiglitazone is neuroprotective after traumatic brain injury via anti-inflammatory and anti-oxidative mechanisms. Brain Res. 2008, 1244:164-172.
41. Zhao W., Varghese M., Yemul S., Pan Y., Cheng A., Marano P., Hassan S., Vempati P., Chen F., Qian X., Pasinetti G.M. Peroxisome proliferator activator receptor gamma coactivator-1alpha (PGC-1alpha) improves motor performance and survival in a mouse model of amyotrophic lateral sclerosis. Mol. Neurodegener. 2011, 6:51.