PPARgamma rescue of the mitochondrial dysfunction in Huntington's disease

Neurobiology of Disease

Volumn 45, Issue 1, 2012, Pages 322-328

  Chiang, Ming Chang ^a   Chern, Yijuang ^b   Huang, Rong Nan ^c  

^a CHINESE CULTURE UNIVERSITY   (Taiwan)

^b INSTITUTE OF BIOMEDICAL SCIENCES   (Taiwan)

^c NATIONAL TAIWAN UNIVERSITY   (Taiwan)

Author keywords

Huntingtin; Mitochondrial function; PGC 1 ; PPAR ; Thiazolidinedione

Indexed keywords

2,4 THIAZOLIDINEDIONE DERIVATIVE; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR GAMMA; ROSIGLITAZONE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BRAIN CORTEX; CONTROLLED STUDY; DOWN REGULATION; DRUG EFFICACY; GENE; GENE EXPRESSION; GENE FUNCTION; GENE TARGETING; HUNTINGTON CHOREA; MALE; MITOCHONDRIAL GENE; MOUSE; NEUROPROTECTION; NONHUMAN; PGC 1A GENE; PRIORITY JOURNAL; RECEPTOR BLOCKING; UPREGULATION;

ANIMALS; CEREBRAL CORTEX; DISEASE MODELS, ANIMAL; DOWN-REGULATION; ENERGY METABOLISM; HUNTINGTON DISEASE; MALE; MICE; MICE, TRANSGENIC; MITOCHONDRIA; PPAR GAMMA; REACTIVE OXYGEN SPECIES; THIAZOLIDINEDIONES; TRINUCLEOTIDE REPEAT EXPANSION;

EID: 81955162873     PISSN: 09699961     EISSN: 1095953X     Source Type: Journal    
DOI: 10.1016/j.nbd.2011.08.016     Document Type: Article

References (52)

1. Bordet R., et al. PPAR: a new pharmacological target for neuroprotection in stroke and neurodegenerative diseases. Biochem. Soc. Trans. 2006, 34:1341-1346.
2. Busch A., et al. Mutant huntingtin promotes the fibrillogenesis of wild-type huntingtin: a potential mechanism for loss of huntingtin function in Huntington's disease. J. Biol. Chem. 2003, 278:41452-41461.
3. Cepeda C., et al. The corticostriatal pathway in Huntington's disease. Prog. Neurobiol. 2007, 81:253-271.
4. Chang D.T., et al. Mutant huntingtin aggregates impair mitochondrial movement and trafficking in cortical neurons. Neurobiol. Dis. 2006, 22:388-400.
5. Chaturvedi R.K., et al. Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation. Hum. Mol. Genet. 2010, 19:3190-3205.
6. Chiang M.C., et al. cAMP-response element-binding protein contributes to suppression of the A2A adenosine receptor promoter by mutant Huntingtin with expanded polyglutamine residues. J. Biol. Chem. 2005, 280:14331-14340.
7. Chiang M.C., et al. Dysregulation of C/EBPalpha by mutant Huntingtin causes the urea cycle deficiency in Huntington's disease. Hum. Mol. Genet. 2007, 16:483-498.
8. Chiang M.C., et al. Systematic uncovering of multiple pathways underlying the pathology of Huntington disease by an acid-cleavable isotope-coded affinity tag approach. Mol. Cell. Proteomics 2007, 6:781-797.
9. Chiang M.C., et al. The A2A adenosine receptor rescues the urea cycle deficiency of Huntington's disease by enhancing the activity of the ubiquitin-proteasome system. Hum. Mol. Genet. 2009, 18:2929-2942.
10. Chiang M.C., et al. Modulation of energy deficiency in Huntington's disease via activation of the peroxisome proliferator-activated receptor gamma. Hum. Mol. Genet. 2010, 19:4043-4058.
11. Chiang M.C., et al. The dysfunction of hepatic transcriptional factors in mice with Huntington's disease. Biochim. Biophys. Acta 2011, 1812:1111-1120.
12. Combs C.K., et al. Inflammatory mechanisms in Alzheimer's disease: inhibition of beta-amyloid-stimulated proinflammatory responses and neurotoxicity by PPARgamma agonists. J. Neurosci. 2000, 20:558-567.
13. Cui L., et al. Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell 2006, 127:59-69.
14. Cummings D.M., et al. Alterations in cortical excitation and inhibition in genetic mouse models of Huntington's disease. J. Neurosci. 2009, 29:10371-10386.
15. Damiano M., et al. Mitochondria in Huntington's disease. Biochim. Biophys. Acta 2010, 1802:52-61.
16. Davies S.W., et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 1997, 90:537-548.
17. Dunah A.W., et al. Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease. Science 2002, 296:2238-2243.
18. Fajas L., et al. The organization, promoter analysis, and expression of the human PPARgamma gene. J. Biol. Chem. 1997, 272:18779-18789.
19. Fuenzalida K., et al. Peroxisome proliferator-activated receptor gamma up-regulates the Bcl-2 anti-apoptotic protein in neurons and induces mitochondrial stabilization and protection against oxidative stress and apoptosis. J. Biol. Chem. 2007, 282:37006-37015.
20. Ghosh S., et al. The thiazolidinedione pioglitazone alters mitochondrial function in human neuron-like cells. Mol. Pharmacol. 2007, 71:1695-1702.
21. Guan H.P., et al. Corepressors selectively control the transcriptional activity of PPARgamma in adipocytes. Genes Dev. 2005, 19:453-461.
22. Hathorn T., et al. Nicotinamide improves motor deficits and upregulates PGC-1alpha and BDNF gene expression in a mouse model of Huntington's disease. Neurobiol. Dis. 2010, 41:43-50.
23. Hondares E., et al. Thiazolidinediones and rexinoids induce peroxisome proliferator-activated receptor-coactivator (PGC)-1alpha gene transcription: an autoregulatory loop controls PGC-1alpha expression in adipocytes via peroxisome proliferator-activated receptor-gamma coactivation. Endocrinology 2006, 147:2829-2838.
24. Imarisio S., et al. Huntington's disease: from pathology and genetics to potential therapies. Biochem. J. 2008, 412:191-209.
25. Kiaei M., et al. Peroxisome proliferator-activated receptor-gamma agonist extends survival in transgenic mouse model of amyotrophic lateral sclerosis. Exp. Neurol. 2005, 191:331-336.
26. Laemmli U.K. Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature 1970, 227:680-685.
27. Landreth G. PPARgamma agonists as new therapeutic agents for the treatment of Alzheimer's disease. Exp. Neurol. 2006, 199:245-248.
28. Lehrke M., Lazar M.A. The many faces of PPARgamma. Cell 2005, 123:993-999.
29. Mangiarini L., et al. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 1996, 87:493-506.
30. Martin J.B., Gusella J.F. Huntington's disease. Pathogenesis and management. N Engl J. Med. 1986, 315:1267-1276.
31. McGill J.K., Beal M.F. PGC-1alpha, a new therapeutic target in Huntington's disease?. Cell 2006, 127:465-468.
32. Milakovic T., Johnson G.V. Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin. J. Biol. Chem. 2005, 280:30773-30782.
33. Pagel-Langenickel I., et al. PGC-1alpha integrates insulin signaling, mitochondrial regulation, and bioenergetic function in skeletal muscle. J. Biol. Chem. 2008, 283:22464-22472.
34. Panov A.V., et al. Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat. Neurosci. 2002, 5:731-736.
35. Pendergrass W., et al. Efficacy of MitoTracker Green and CMXrosamine to measure changes in mitochondrial membrane potentials in living cells and tissues. Cytom. A 2004, 61:162-169.
36. Perez-Severiano F., et al. Increased formation of reactive oxygen species, but no changes in glutathione peroxidase activity, in striata of mice transgenic for the Huntington's disease mutation. Neurochem. Res. 2004, 29:729-733.
37. Puigserver P., Spiegelman B.M. Peroxisome proliferator-activated receptor-gamma coactivator 1 alpha (PGC-1 alpha): transcriptional coactivator and metabolic regulator. Endocr. Rev. 2003, 24:78-90.
38. Quintanilla R.A., et al. Rosiglitazone treatment prevents mitochondrial dysfunction in mutant huntingtin-expressing cells: possible role of peroxisome proliferator-activated receptor-gamma (PPARgamma) in the pathogenesis of Huntington disease. J. Biol. Chem. 2008, 283:25628-25637.
39. Reddy P.H., et al. Recent advances in understanding the pathogenesis of Huntington's disease. Trends Neurosci. 1999, 22:248-255.
40. Rego A.C., Oliveira C.R. Mitochondrial dysfunction and reactive oxygen species in excitotoxicity and apoptosis: implications for the pathogenesis of neurodegenerative diseases. Neurochem. Res. 2003, 28:1563-1574.
41. Scarpulla R.C. Nuclear control of respiratory gene expression in mammalian cells. J. Cell. Biochem. 2006, 97:673-683.
42. Seong I.S., et al. HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism. Hum. Mol. Genet. 2005, 14:2871-2880.
43. Steffan J.S., et al. The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc. Natl Acad. Sci. USA 2000, 97:6763-6768.
44. St-Pierre J., et al. Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators. Cell 2006, 127:397-408.
45. Sundararajan S., et al. PPARgamma as a therapeutic target in central nervous system diseases. Neurochem. Int. 2006, 49:136-144.
46. Vonsattel J.P., et al. Neuropathological classification of Huntington's disease. J. Neuropathol. Exp. Neurol. 1985, 44:559-577.
47. Walker G.E., et al. Subcutaneous abdominal adipose tissue subcompartments: potential role in rosiglitazone effects. Obesity (Silver Spring) 2008, 16:1983-1991.
48. Wareski P., et al. PGC-1{alpha} and PGC-1{beta} regulate mitochondrial density in neurons. J. Biol. Chem. 2009, 284:21379-21385.
49. Wilson-Fritch L., et al. Mitochondrial biogenesis and remodeling during adipogenesis and in response to the insulin sensitizer rosiglitazone. Mol. Cell. Biol. 2003, 23:1085-1094.
50. Wu Z., et al. Mechanisms controlling mitochondrial biogenesis and respiration through the thermogenic coactivator PGC-1. Cell 1999, 98:115-124.
51. Wyttenbach A., et al. Heat shock protein 27 prevents cellular polyglutamine toxicity and suppresses the increase of reactive oxygen species caused by huntingtin. Hum. Mol. Genet. 2002, 11:1137-1151.
52. Yu Z.X., et al. Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease. J. Neurosci. 2003, 23:2193-2202.