The dysfunction of hepatic transcriptional factors in mice with Huntington's Disease

Biochimica et Biophysica Acta - Molecular Basis of Disease

Volumn 1812, Issue 9, 2011, Pages 1111-1120

  Chiang, Ming Chang ^a   Chern, Yijuang ^b   Juo, Chiun Gung ^c  

^a CHINESE CULTURE UNIVERSITY   (Taiwan)

^b INSTITUTE OF BIOMEDICAL SCIENCES   (Taiwan)

^c CHANG GUNG UNIVERSITY   (Taiwan)

Author keywords

C EBP ; Huntington's Disease; Mitochondrial function; PGC 1 ; PPAR ; Thiazolidinedione

Indexed keywords

2 CHLORO 5 NITROBENZANILIDE; 2,4 THIAZOLIDINEDIONE DERIVATIVE; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR GAMMA; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR GAMMA COACTIVATOR 1ALPHA; ROSIGLITAZONE; TRANSCRIPTION FACTOR;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; CELL STRAIN HEPG2; CONTROLLED STUDY; DOWN REGULATION; GENE; GENE EXPRESSION; HEPATOMA CELL; HUNTINGTON CHOREA; LIVER DYSFUNCTION; LIVER PROTECTION; MALE; MITOCHONDRION; MOUSE; NONHUMAN; PRIORITY JOURNAL;

ANIMALS; HUNTINGTON DISEASE; LIVER; MICE; MICE, TRANSGENIC; NERVE TISSUE PROTEINS; NUCLEAR PROTEINS; PPAR GAMMA; THIAZOLIDINEDIONES; TRANS-ACTIVATORS; TRANSCRIPTION FACTORS;

MUS; MUS MUSCULUS;

EID: 79960564783     PISSN: 09254439     EISSN: 1879260X     Source Type: Journal    
DOI: 10.1016/j.bbadis.2011.05.006     Document Type: Article

References (81)

1. Martin J.B., Gusella J.F. Huntington's disease. Pathogenesis and management. N. Engl. J. Med. 1986, 315:1267-1276.
2. Vonsattel J.P., Myers R.H., Stevens T.J., Ferrante R.J., Bird E.D., Richardson E.P. Neuropathological classification of Huntington's disease. J. Neuropathol. Exp. Neurol. 1985, 44:559-577.
3. MacDonald M. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group. Cell 1993, 72:971-983.
4. Reddy P.H., Williams M., Tagle D.A. Recent advances in understanding the pathogenesis of Huntington's disease. Trends Neurosci. 1999, 22:248-255.
5. Podolsky S., Leopold N.A., Sax D.S. Increased frequency of diabetes mellitus in patients with Huntington's chorea. Lancet 1972, 1:1356-1358.
6. Djousse L., Knowlton B., Cupples L.A., Marder K., Shoulson I., Myers R.H. Weight loss in early stage of Huntington's disease. Neurology 2002, 59:1325-1330.
7. Menkes J.H., Stein N. Fibroblast cultures in Huntington's disease. N. Engl. J. Med. 1973, 288:856-857.
8. McGovern D., Webb T. Sensitivity to ionising radiation of lymphocytes from Huntington's chorea patients compared to controls. J. Med. Genet. 1982, 19:168-174.
9. Hurlbert M.S., Zhou W., Wasmeier C., Kaddis F.G., Hutton J.C., Freed C.R. Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes. Diabetes 1999, 48:649-651.
10. Chaturvedi R.K., Adhihetty P., Shukla S., Hennessy T., Calingasan N., Yang L., Starkov A., Kiaei M., Cannella M., Sassone J., Ciammola A., Squitieri F., Beal M.F. Impaired PGC-1alpha function in muscle in Huntington's disease. Hum. Mol. Genet. 2009, 18:3048-3065.
11. Busse M.E., Hughes G., Wiles C.M., Rosser A.E. Use of hand-held dynamometry in the evaluation of lower limb muscle strength in people with Huntington's disease. J. Neurol. 2008, 255:1534-1540.
12. Phan J., Hickey M.A., Zhang P., Chesselet M.F., Reue K. Adipose tissue dysfunction tracks disease progression in two Huntington's disease mouse models. Hum. Mol. Genet. 2009, 18:1006-1016.
13. Karpuj M.V., Becher M.W., Springer J.E., Chabas D., Youssef S., Pedotti R., Mitchell D., Steinman L. Prolonged survival and decreased abnormal movements in transgenic model of Huntington disease, with administration of the transglutaminase inhibitor cystamine. Nat. Med. 2002, 8:143-149.
14. Sathasivam K., Hobbs C., Turmaine M., Mangiarini L., Mahal A., Bertaux F., Wanker E.E., Doherty P., Davies S.W., Bates G.P. Formation of polyglutamine inclusions in non-CNS tissue. Hum. Mol. Genet. 1999, 8:813-822.
15. Tanaka M., Machida Y., Niu S., Ikeda T., Jana N.R., Doi H., Kurosawa M., Nekooki M., Nukina N. Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease. Nat. Med. 2004, 10:148-154.
16. Chiang M.C., Chen H.M., Lee Y.H., Chang H.H., Wu Y.C., Soong B.W., Chen C.M., Wu Y.R., Liu C.S., Niu D.M., Wu J.Y., Chen Y.T., Chern Y. Dysregulation of C/EBPalpha by mutant Huntingtin causes the urea cycle deficiency in Huntington's disease. Hum. Mol. Genet. 2007, 16:483-498.
17. Valenza M., Carroll J.B., Leoni V., Bertram L.N., Bjorkhem I., Singaraja R.R., Di Donato S., Lutjohann D., Hayden M.R., Cattaneo E. Cholesterol biosynthesis pathway is disturbed in YAC128 mice and is modulated by huntingtin mutation. Hum. Mol. Genet. 2007, 16:2187-2198.
18. Panov A.V., Gutekunst C.A., Leavitt B.R., Hayden M.R., Burke J.R., Strittmatter W.J., Greenamyre J.T. Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat. Neurosci. 2002, 5:731-736.
19. Cha J.H. Transcriptional signatures in Huntington's disease. Prog. Neurobiol. 2007, 83:228-248.
20. Chiang M.C., Lee Y.C., Huang C.L., Chern Y. 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.
21. Chiang M.C., Juo C.G., Chang H.H., Chen H.M., Yi E.C., Chern Y. 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.
22. Dunah A.W., Jeong H., Griffin A., Kim Y.M., Standaert D.G., Hersch S.M., Mouradian M.M., Young A.B., Tanese N., Krainc D. Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease. Science 2002, 296:2238-2243.
23. Grunewald T., Beal M.F. Bioenergetics in Huntington's disease. Ann. N. Y. Acad. Sci. 1999, 893:203-213.
24. van der Burg J.M., Bjorkqvist M., Brundin P. Beyond the brain: widespread pathology in Huntington's disease. Lancet Neurol. 2009, 8:765-774.
25. Rosen E.D., Hsu C.H., Wang X., Sakai S., Freeman M.W., Gonzalez F.J., Spiegelman B.M. C/EBPalpha induces adipogenesis through PPARgamma: a unified pathway. Genes Dev. 2002, 16:22-26.
26. Chiang M.C., Chen C.M., Lee M.R., Chen H.W., Chen H.M., Wu Y.S., Hung C.H., Kang J.J., Chang C.P., Chang C., Wu Y.R., Tsai Y.S., Chern Y. Modulation of energy deficiency in Huntington's disease via activation of the peroxisome proliferator-activated receptor gamma. Hum. Mol. Genet. 2010, 19:4043-4058.
27. 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.
28. Cui L., Jeong H., Borovecki F., Parkhurst C.N., Tanese N., Krainc D. Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell 2006, 127:59-69.
29. Weydt P., Pineda V.V., Torrence A.E., Libby R.T., Satterfield T.F., Lazarowski E.R., Gilbert M.L., Morton G.J., Bammler T.K., Strand A.D., Cui L., Beyer R.P., Easley C.N., Smith A.C., Krainc D., Luquet S., Sweet I.R., Schwartz M.W., La Spada A.R. Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration. Cell Metab. 2006, 4:349-362.
30. Lehrke M., Lazar M.A. The many faces of PPARgamma. Cell 2005, 123:993-999.
31. Quintanilla R.A., Jin Y.N., Fuenzalida K., Bronfman M., Johnson G.V. 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.
32. Kim H.I., Koh Y.K., Kim T.H., Kwon S.K., Im S.S., Choi H.S., Kim K.S., Ahn Y.H. Transcriptional activation of SHP by PPAR-gamma in liver. Biochem. Biophys. Res. Commun. 2007, 360:301-306.
33. Kim S.Y., Kim H.I., Park S.K., Im S.S., Li T., Cheon H.G., Ahn Y.H. Liver glucokinase can be activated by peroxisome proliferator-activated receptor-gamma. Diabetes 2004, 53(Suppl. 1):S66-S70.
34. Chiang M.C., Chen H.M., Lai H.L., Chen H.W., Chou S.Y., Chen C.M., Tsai F.J., Chern Y. 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.
35. Laemmli U.K. Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature 1970, 227:680-685.
36. Want E.J., Wilson I.D., Gika H., Theodoridis G., Plumb R.S., Shockcor J., Holmes E., Nicholson J.K. Global metabolic profiling procedures for urine using UPLC-MS. Nat. Protoc. 2010, 5:1005-1018.
37. Chaturvedi R.K., Calingasan N.Y., Yang L., Hennessey T., Johri A., Beal M.F. 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.
38. Ho D.J., Calingasan N.Y., Wille E., Dumont M., Beal M.F. Resveratrol protects against peripheral deficits in a mouse model of Huntington's disease. Exp. Neurol. 2010, 225:74-84.
39. Hondares E., Mora O., Yubero P., Rodriguez de la Concepcion M., Iglesias R., Giralt M., Villarroya F. 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.
40. Guan H.P., Ishizuka T., Chui P.C., Lehrke M., Lazar M.A. Corepressors selectively control the transcriptional activity of PPARgamma in adipocytes. Genes Dev. 2005, 19:453-461.
41. McGill J.K., Beal M.F. PGC-1alpha, a new therapeutic target in Huntington's disease?. Cell 2006, 127:465-468.
42. Ghosh S., Patel N., Rahn D., McAllister J., Sadeghi S., Horwitz G., Berry D., Wang K.X., Swerdlow R.H. The thiazolidinedione pioglitazone alters mitochondrial function in human neuron-like cells. Mol. Pharmacol. 2007, 71:1695-1702.
43. Tanaka T., Yamamoto J., Iwasaki S., Asaba H., Hamura H., Ikeda Y., Watanabe M., Magoori K., Ioka R.X., Tachibana K., Watanabe Y., Uchiyama Y., Sumi K., Iguchi H., Ito S., Doi T., Hamakubo T., Naito M., Auwerx J., Yanagisawa M., Kodama T., Sakai J. Activation of peroxisome proliferator-activated receptor delta induces fatty acid beta-oxidation in skeletal muscle and attenuates metabolic syndrome. Proc. Natl Acad. Sci. U. S. A. 2003, 100:15924-15929.
44. 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.
45. Kubota N., Terauchi Y., Miki H., Tamemoto H., Yamauchi T., Komeda K., Satoh S., Nakano R., Ishii C., Sugiyama T., Eto K., Tsubamoto Y., Okuno A., Murakami K., Sekihara H., Hasegawa G., Naito M., Toyoshima Y., Tanaka S., Shiota K., Kitamura T., Fujita T., Ezaki O., Aizawa S., Kadowaki T., et al. PPAR gamma mediates high-fat diet-induced adipocyte hypertrophy and insulin resistance. Mol. Cell 1999, 4:597-609.
46. Ren D., Collingwood T.N., Rebar E.J., Wolffe A.P., Camp H.S. PPARgamma knockdown by engineered transcription factors: exogenous PPARgamma2 but not PPARgamma1 reactivates adipogenesis. Genes Dev. 2002, 16:27-32.
47. Fajas L., Auboeuf D., Raspe E., Schoonjans K., Lefebvre A.M., Saladin R., Najib J., Laville M., Fruchart J.C., Deeb S., Vidal-Puig A., Flier J., Briggs M.R., Staels B., Vidal H., Auwerx J. The organization, promoter analysis, and expression of the human PPARgamma gene. J. Biol. Chem. 1997, 272:18779-18789.
48. Bordet R., Ouk T., Petrault O., Gele P., Gautier S., Laprais M., Deplanque D., Duriez P., Staels B., Fruchart J.C., Bastide M. PPAR: a new pharmacological target for neuroprotection in stroke and neurodegenerative diseases. Biochem. Soc. Trans. 2006, 34:1341-1346.
49. Desvergne B., Michalik L., Wahli W. Transcriptional regulation of metabolism. Physiol. Rev. 2006, 86:465-514.
50. Way J.M., Harrington W.W., Brown K.K., Gottschalk W.K., Sundseth S.S., Mansfield T.A., Ramachandran R.K., Willson T.M., Kliewer S.A. Comprehensive messenger ribonucleic acid profiling reveals that peroxisome proliferator-activated receptor gamma activation has coordinate effects on gene expression in multiple insulin-sensitive tissues. Endocrinology 2001, 142:1269-1277.
51. Beal M.F. Mitochondria, free radicals, and neurodegeneration. Curr. Opin. Neurobiol. 1996, 6:661-666.
52. Sawa A., Wiegand G.W., Cooper J., Margolis R.L., Sharp A.H., Lawler J.F., Greenamyre J.T., Snyder S.H., Ross C.A. Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization. Nat. Med. 1999, 5:1194-1198.
53. Beal M.F. Energetics in the pathogenesis of neurodegenerative diseases. Trends Neurosci. 2000, 23:298-304.
54. Browne S.E., Beal M.F. The energetics of Huntington's disease. Neurochem. Res. 2004, 29:531-546.
55. Damiano M., Galvan L., Deglon N., Brouillet E. Mitochondria in Huntington's disease. Biochim. Biophys. Acta 2010, 1802:52-61.
56. Mann V.M., Cooper J.M., Javoy-Agid F., Agid Y., Jenner P., Schapira A.H. Mitochondrial function and parental sex effect in Huntington's disease. Lancet 1990, 336:749.
57. 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.
58. Seong I.S., Ivanova E., Lee J.M., Choo Y.S., Fossale E., Anderson M., Gusella J.F., Laramie J.M., Myers R.H., Lesort M., MacDonald M.E. HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism. Hum. Mol. Genet. 2005, 14:2871-2880.
59. Fuenzalida K., Quintanilla R., Ramos P., Piderit D., Fuentealba R.A., Martinez G., Inestrosa N.C., Bronfman M. 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.
60. Vidal-Puig A.J., Considine R.V., Jimenez-Linan M., Werman A., Pories W.J., Caro J.F., Flier J.S. Peroxisome proliferator-activated receptor gene expression in human tissues. Effects of obesity, weight loss, and regulation by insulin and glucocorticoids. J. Clin. Invest. 1997, 99:2416-2422.
61. Hathorn T., Snyder-Keller A., Messer A. 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.
62. Larsson N.G., Wang J., Wilhelmsson H., Oldfors A., Rustin P., Lewandoski M., Barsh G.S., Clayton D.A. Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice. Nat. Genet. 1998, 18:231-236.
63. Scarpulla R.C. Nuclear control of respiratory gene expression in mammalian cells. J. Cell. Biochem. 2006, 97:673-683.
64. Lin J., Handschin C., Spiegelman B.M. Metabolic control through the PGC-1 family of transcription coactivators. Cell Metab. 2005, 1:361-370.
65. Everett L., Galli A., Crabb D. The role of hepatic peroxisome proliferator-activated receptors (PPARs) in health and disease. Liver 2000, 20:191-199.
66. Koo S.H., Satoh H., Herzig S., Lee C.H., Hedrick S., Kulkarni R., Evans R.M., Olefsky J., Montminy M. PGC-1 promotes insulin resistance in liver through PPAR-alpha-dependent induction of TRB-3. Nat. Med. 2004, 10:530-534.
67. Lee S.S., Pineau T., Drago J., Lee E.J., Owens J.W., Kroetz D.L., Fernandez-Salguero P.M., Westphal H., Gonzalez F.J. Targeted disruption of the alpha isoform of the peroxisome proliferator-activated receptor gene in mice results in abolishment of the pleiotropic effects of peroxisome proliferators. Mol. Cell. Biol. 1995, 15:3012-3022.
68. Birkenmeier E.H., Gwynn B., Howard S., Jerry J., Gordon J.I., Landschulz W.H., McKnight S.L. Tissue-specific expression, developmental regulation, and genetic mapping of the gene encoding CCAAT/enhancer binding protein. Genes Dev. 1989, 3:1146-1156.
69. Kimura T., Christoffels V.M., Chowdhury S., Iwase K., Matsuzaki H., Mori M., Lamers W.H., Darlington G.J., Takiguchi M. Hypoglycemia-associated hyperammonemia caused by impaired expression of ornithine cycle enzyme genes in C/EBPalpha knockout mice. J. Biol. Chem. 1998, 273:27505-27510.
70. Inoue Y., Inoue J., Lambert G., Yim S.H., Gonzalez F.J. Disruption of hepatic C/EBPalpha results in impaired glucose tolerance and age-dependent hepatosteatosis. J. Biol. Chem. 2004, 279:44740-44748.
71. Moffitt H., McPhail G.D., Woodman B., Hobbs C., Bates G.P. Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease. PLoS One 2009, 4:e8025.
72. Endo F., Matsuura T., Yanagita K., Matsuda I. Clinical manifestations of inborn errors of the urea cycle and related metabolic disorders during childhood. J. Nutr. 2004, 134:1605S-1609S. discussion 1630S-1632S, 1667S-1672S.
73. Bolt J.M., Lewis G.P. Huntington's chorea. A study of liver function and histology. Q. J. Med. 1973, 42:151-174.
74. Chiu E., Mackay I.R., Bhathal P.B. Hepatic morphology in Huntington's chorea. J. Neurol. Neurosurg. Psychiatry 1975, 38:1000-1002.
75. Maywood E.S., Fraenkel E., McAllister C.J., Wood N., Reddy A.B., Hastings M.H., Morton A.J. Disruption of peripheral circadian timekeeping in a mouse model of Huntington's disease and its restoration by temporally scheduled feeding. J. Neurosci. 2010, 30:10199-10204.
76. Picker J.D., Puga A.C., Levy H.L., Marsden D., Shih V.E., Degirolami U., Ligon K.L., Cederbaum S.D., Kern R.M., Cox G.F. Arginase deficiency with lethal neonatal expression: evidence for the glutamine hypothesis of cerebral edema. J. Pediatr. 2003, 142:349-352.
77. Deignan J.L., Livesay J.C., Yoo P.K., Goodman S.I., O'Brien W.E., Iyer R.K., Cederbaum S.D., Grody W.W. Ornithine deficiency in the arginase double knockout mouse. Mol. Genet. Metab. 2006, 89:87-96.
78. Gutekunst C.A., Norflus F., Hersch S.M. Recent advances in Huntington's disease. Curr. Opin. Neurol. 2000, 13:445-450.
79. Felipo V., Butterworth R.F. Mitochondrial dysfunction in acute hyperammonemia. Neurochem. Int. 2002, 40:487-491.
80. Szerb J.C., Butterworth R.F. Effect of ammonium ions on synaptic transmission in the mammalian central nervous system. Prog. Neurobiol. 1992, 39:135-153.
81. Martin B., Golden E., Keselman A., Stone M., Mattson M.P., Egan J.M., Maudsley S. Therapeutic perspectives for the treatment of Huntington's disease: treating the whole body. Histol. Histopathol. 2008, 23:237-250.