{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,14]],"date-time":"2026-09-14T03:21:53Z","timestamp":1789356113006,"version":"build-2803163510"},"reference-count":74,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2010,6,1]],"date-time":"2010-06-01T00:00:00Z","timestamp":1275350400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Bioenerg Biomembr"],"published-print":{"date-parts":[[2010,6]]},"DOI":"10.1007\/s10863-010-9286-7","type":"journal-article","created":{"date-parts":[[2010,6,16]],"date-time":"2010-06-16T02:24:19Z","timestamp":1276655059000},"page":"199-205","source":"Crossref","is-referenced-by-count":70,"title":["The interrelationship between mitochondrial dysfunction and transcriptional dysregulation in Huntington disease"],"prefix":"10.1007","volume":"42","author":[{"given":"Youngnam N.","family":"Jin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gail V. W.","family":"Johnson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2010,6,17]]},"reference":[{"issue":"1","key":"9286_CR1","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1016\/j.dnarep.2008.09.004","volume":"8","author":"K Acevedo-Torres","year":"2009","unstructured":"Acevedo-Torres K, Berrios L, Rosario N, Dufault V, Skatchkov S, Eaton MJ, Torres-Ramos CA, Ayala-Torres S (2009) Mitochondrial DNA damage is a hallmark of chemically induced and the R6\/2 transgenic model of Huntington\u2019s disease. DNA Repair (Amst) 8(1):126\u2013136","journal-title":"DNA Repair (Amst)"},{"issue":"3","key":"9286_CR2","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1002\/ana.410430321","volume":"43","author":"J Arenas","year":"1998","unstructured":"Arenas J, Campos Y, Ribacoba R, Martin MA, Rubio JC, Ablanedo P, Cabello A (1998) Complex I defect in muscle from patients with Huntington\u2019s disease. Ann Neurol 43(3):397\u2013400","journal-title":"Ann Neurol"},{"issue":"3","key":"9286_CR3","doi-asserted-by":"crossref","first-page":"1147","DOI":"10.1111\/j.1471-4159.1993.tb03633.x","volume":"61","author":"MF Beal","year":"1993","unstructured":"Beal MF, Brouillet E, Jenkins B, Henshaw R, Rosen B, Hyman BT (1993) Age-dependent striatal excitotoxic lesions produced by the endogenous mitochondrial inhibitor malonate. J Neurochem 61(3):1147\u20131150","journal-title":"J Neurochem"},{"issue":"4","key":"9286_CR4","doi-asserted-by":"crossref","first-page":"1652","DOI":"10.1091\/mbc.e05-07-0607","volume":"17","author":"A Benchoua","year":"2006","unstructured":"Benchoua A, Trioulier Y, Zala D, Gaillard MC, Lefort N, Dufour N, Saudou F, Elalouf JM, Hirsch E, Hantraye P et al (2006) Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin. Mol Biol Cell 17(4):1652\u20131663","journal-title":"Mol Biol Cell"},{"issue":"3","key":"9286_CR5","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1046\/j.1471-4159.2002.00990.x","volume":"82","author":"T Breidert","year":"2002","unstructured":"Breidert T, Callebert J, Heneka MT, Landreth G, Launay JM, Hirsch EC (2002) Protective action of the peroxisome proliferator-activated receptor-gamma agonist pioglitazone in a mouse model of Parkinson\u2019s disease. J Neurochem 82(3):615\u2013624","journal-title":"J Neurochem"},{"issue":"1","key":"9286_CR6","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1111\/j.1471-4159.1993.tb05859.x","volume":"60","author":"E Brouillet","year":"1993","unstructured":"Brouillet E, Jenkins BG, Hyman BT, Ferrante RJ, Kowall NW, Srivastava R, Roy DS, Rosen BR, Beal MF (1993) Age-dependent vulnerability of the striatum to the mitochondrial toxin 3-nitropropionic acid. J Neurochem 60(1):356\u2013359","journal-title":"J Neurochem"},{"issue":"12","key":"9286_CR7","doi-asserted-by":"crossref","first-page":"4858","DOI":"10.1523\/JNEUROSCI.23-12-04858.2003","volume":"23","author":"N Brustovetsky","year":"2003","unstructured":"Brustovetsky N, Brustovetsky T, Purl KJ, Capano M, Crompton M, Dubinsky JM (2003) Increased susceptibility of striatal mitochondria to calcium-induced permeability transition. J Neurosci 23(12):4858\u20134867","journal-title":"J Neurosci"},{"issue":"2","key":"9286_CR8","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1016\/0022-510X(85)90112-1","volume":"67","author":"J Butterworth","year":"1985","unstructured":"Butterworth J, Yates CM, Reynolds GP (1985) Distribution of phosphate-activated glutaminase, succinic dehydrogenase, pyruvate dehydrogenase and gamma-glutamyl transpeptidase in post-mortem brain from Huntington\u2019s disease and agonal cases. J Neurol Sci 67(2):161\u2013171","journal-title":"J Neurol Sci"},{"issue":"5\u20136","key":"9286_CR9","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1016\/j.pneurobio.2006.11.001","volume":"81","author":"C Cepeda","year":"2007","unstructured":"Cepeda C, Wu N, Andre VM, Cummings DM, Levine MS (2007) The corticostriatal pathway in Huntington\u2019s disease. Prog Neurobiol 81(5\u20136):253\u2013271","journal-title":"Prog Neurobiol"},{"issue":"14","key":"9286_CR10","doi-asserted-by":"crossref","first-page":"1407","DOI":"10.1093\/hmg\/ddh162","volume":"13","author":"YS Choo","year":"2004","unstructured":"Choo YS, Johnson GV, MacDonald M, Detloff PJ, Lesort M (2004) Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release. Hum Mol Genet 13(14):1407\u20131420","journal-title":"Hum Mol Genet"},{"issue":"1","key":"9286_CR11","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.cell.2006.09.015","volume":"127","author":"L Cui","year":"2006","unstructured":"Cui L, Jeong H, Borovecki F, Parkhurst CN, Tanese N, Krainc D (2006) Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell 127(1):59\u201369","journal-title":"Cell"},{"issue":"1","key":"9286_CR12","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.bbadis.2009.07.012","volume":"1802","author":"M Damiano","year":"2010","unstructured":"Damiano M, Galvan L, Deglon N, Brouillet E (2010) Mitochondria in Huntington\u2019s disease. Biochim Biophys Acta 1802(1):52\u201361","journal-title":"Biochim Biophys Acta"},{"issue":"3","key":"9286_CR13","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1016\/S0092-8674(00)80513-9","volume":"90","author":"SW Davies","year":"1997","unstructured":"Davies SW, Turmaine M, Cozens BA, DiFiglia M, Sharp AH, Ross CA, Scherzinger E, Wanker EE, Mangiarini L, Bates GP (1997) Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90(3):537\u2013548","journal-title":"Cell"},{"issue":"5334","key":"9286_CR14","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1126\/science.277.5334.1990","volume":"277","author":"M DiFiglia","year":"1997","unstructured":"DiFiglia M, Sapp E, Chase KO, Davies SW, Bates GP, Vonsattel JP, Aronin N (1997) Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 277(5334):1990\u20131993","journal-title":"Science"},{"issue":"9","key":"9286_CR15","doi-asserted-by":"crossref","first-page":"1325","DOI":"10.1212\/01.WNL.0000031791.10922.CF","volume":"59","author":"L Djousse","year":"2002","unstructured":"Djousse L, Knowlton B, Cupples LA, Marder K, Shoulson I, Myers RH (2002) Weight loss in early stage of Huntington\u2019s disease. Neurology 59(9):1325\u20131330","journal-title":"Neurology"},{"issue":"5576","key":"9286_CR16","doi-asserted-by":"crossref","first-page":"2238","DOI":"10.1126\/science.1072613","volume":"296","author":"AW Dunah","year":"2002","unstructured":"Dunah AW, Jeong H, Griffin A, Kim YM, Standaert DG, Hersch SM, Mouradian MM, Young AB, Tanese N, Krainc D (2002) Sp1 and TAFII130 transcriptional activity disrupted in early Huntington\u2019s disease. Science 296(5576):2238\u20132243","journal-title":"Science"},{"issue":"14","key":"9286_CR17","doi-asserted-by":"crossref","first-page":"3768","DOI":"10.1523\/JNEUROSCI.4356-06.2007","volume":"27","author":"MM Fan","year":"2007","unstructured":"Fan MM, Fernandes HB, Zhang LY, Hayden MR, Raymond LA (2007) Altered NMDA receptor trafficking in a yeast artificial chromosome transgenic mouse model of Huntington\u2019s disease. J Neurosci 27(14):3768\u20133779","journal-title":"J Neurosci"},{"issue":"50","key":"9286_CR18","doi-asserted-by":"crossref","first-page":"13614","DOI":"10.1523\/JNEUROSCI.3455-07.2007","volume":"27","author":"HB Fernandes","year":"2007","unstructured":"Fernandes HB, Baimbridge KG, Church J, Hayden MR, Raymond LA (2007) Mitochondrial sensitivity and altered calcium handling underlie enhanced NMDA-induced apoptosis in YAC128 model of Huntington\u2019s disease. J Neurosci 27(50):13614\u201313623","journal-title":"J Neurosci"},{"issue":"12","key":"9286_CR19","doi-asserted-by":"crossref","first-page":"3877","DOI":"10.1523\/JNEUROSCI.11-12-03877.1991","volume":"11","author":"RJ Ferrante","year":"1991","unstructured":"Ferrante RJ, Kowall NW, Richardson EP Jr (1991) Proliferative and degenerative changes in striatal spiny neurons in Huntington\u2019s disease: a combined study using the section-Golgi method and calbindin D28k immunocytochemistry. J Neurosci 11(12):3877\u20133887","journal-title":"J Neurosci"},{"issue":"4","key":"9286_CR20","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1002\/jnr.490350413","volume":"35","author":"DM Frim","year":"1993","unstructured":"Frim DM, Simpson J, Uhler TA, Short MP, Bossi SR, Breakefield XO, Isacson O (1993) Striatal degeneration induced by mitochondrial blockade is prevented by biologically delivered NGF. J Neurosci Res 35(4):452\u2013458","journal-title":"J Neurosci Res"},{"issue":"11","key":"9286_CR21","doi-asserted-by":"crossref","first-page":"2803","DOI":"10.1111\/j.1460-9568.2008.06310.x","volume":"27","author":"JM Gil","year":"2008","unstructured":"Gil JM, Rego AC (2008) Mechanisms of neurodegeneration in Huntington\u2019s disease. Eur J Neurosci 27(11):2803\u20132820","journal-title":"Eur J Neurosci"},{"issue":"5","key":"9286_CR22","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1093\/hmg\/ddg046","volume":"12","author":"S Gines","year":"2003","unstructured":"Gines S, Seong IS, Fossale E, Ivanova E, Trettel F, Gusella JF, Wheeler VC, Persichetti F, MacDonald ME (2003) Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington\u2019s disease knock-in mice. Hum Mol Genet 12(5):497\u2013508","journal-title":"Hum Mol Genet"},{"issue":"2","key":"9286_CR23","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1002\/ana.20754","volume":"59","author":"ZZ Gizatullina","year":"2006","unstructured":"Gizatullina ZZ, Lindenberg KS, Harjes P, Chen Y, Kosinski CM, Landwehrmeyer BG, Ludolph AC, Striggow F, Zierz S, Gellerich FN (2006) Low stability of Huntington muscle mitochondria against Ca2+ in R6\/2 mice. Ann Neurol 59(2):407\u2013411","journal-title":"Ann Neurol"},{"issue":"1","key":"9286_CR24","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1038\/nri1748","volume":"6","author":"CK Glass","year":"2006","unstructured":"Glass CK, Ogawa S (2006) Combinatorial roles of nuclear receptors in inflammation and immunity. Nat Rev Immunol 6(1):44\u201355","journal-title":"Nat Rev Immunol"},{"issue":"1","key":"9286_CR25","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1212\/WNL.28.1.23","volume":"28","author":"HH Goebel","year":"1978","unstructured":"Goebel HH, Heipertz R, Scholz W, Iqbal K, Tellez-Nagel I (1978) Juvenile Huntington chorea: clinical, ultrastructural, and biochemical studies. Neurology 28(1):23\u201331","journal-title":"Neurology"},{"issue":"5","key":"9286_CR26","doi-asserted-by":"crossref","first-page":"518","DOI":"10.1056\/NEJMcibr067022","volume":"356","author":"JT Greenamyre","year":"2007","unstructured":"Greenamyre JT (2007) Huntington\u2019s disease\u2014making connections. N Engl J Med 356(5):518\u2013520","journal-title":"N Engl J Med"},{"issue":"Pt 6","key":"9286_CR27","doi-asserted-by":"crossref","first-page":"1442","DOI":"10.1093\/brain\/awh452","volume":"128","author":"MT Heneka","year":"2005","unstructured":"Heneka MT, Sastre M, Dumitrescu-Ozimek L, Hanke A, Dewachter I, Kuiperi C, O\u2019Banion K, Klockgether T, Van Leuven F, Landreth GE (2005) Acute treatment with the PPARgamma agonist pioglitazone and ibuprofen reduces glial inflammation and Abeta1-42 levels in APPV717I transgenic mice. Brain 128(Pt 6):1442\u20131453","journal-title":"Brain"},{"issue":"10","key":"9286_CR28","doi-asserted-by":"crossref","first-page":"3200","DOI":"10.1523\/JNEUROSCI.5599-08.2009","volume":"29","author":"MY Heng","year":"2009","unstructured":"Heng MY, Detloff PJ, Wang PL, Tsien JZ, Albin RL (2009) In vivo evidence for NMDA receptor-mediated excitotoxicity in a murine genetic model of Huntington disease. J Neurosci 29(10):3200\u20133205","journal-title":"J Neurosci"},{"issue":"10","key":"9286_CR29","doi-asserted-by":"crossref","first-page":"1879","DOI":"10.1212\/WNL.45.10.1879","volume":"45","author":"TM Horton","year":"1995","unstructured":"Horton TM, Graham BH, Corral-Debrinski M, Shoffner JM, Kaufman AE, Beal MF, Wallace DC (1995) Marked increase in mitochondrial DNA deletion levels in the cerebral cortex of Huntington\u2019s disease patients. Neurology 45(10):1879\u20131883","journal-title":"Neurology"},{"issue":"1","key":"9286_CR30","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1016\/j.cell.2004.09.016","volume":"119","author":"SM Houten","year":"2004","unstructured":"Houten SM, Auwerx J (2004) PGC-1alpha: turbocharging mitochondria. Cell 119(1):5\u20137","journal-title":"Cell"},{"issue":"5","key":"9286_CR31","doi-asserted-by":"crossref","first-page":"1357","DOI":"10.1212\/WNL.50.5.1357","volume":"50","author":"BG Jenkins","year":"1998","unstructured":"Jenkins BG, Rosas HD, Chen YC, Makabe T, Myers R, MacDonald M, Rosen BR, Beal MF, Koroshetz WJ (1998) 1H NMR spectroscopy studies of Huntington\u2019s disease: correlations with CAG repeat numbers. Neurology 50(5):1357\u20131365","journal-title":"Neurology"},{"issue":"5","key":"9286_CR32","doi-asserted-by":"crossref","first-page":"e82","DOI":"10.1371\/journal.pgen.0030082","volume":"3","author":"LS Kaltenbach","year":"2007","unstructured":"Kaltenbach LS, Romero E, Becklin RR, Chettier R, Bell R, Phansalkar A, Strand A, Torcassi C, Savage J, Hurlburt A et al (2007) Huntingtin interacting proteins are genetic modifiers of neurodegeneration. PLoS Genet 3(5):e82","journal-title":"PLoS Genet"},{"issue":"2","key":"9286_CR33","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1016\/j.expneurol.2004.10.007","volume":"191","author":"M Kiaei","year":"2005","unstructured":"Kiaei M, Kipiani K, Chen J, Calingasan NY, Beal MF (2005) Peroxisome proliferator-activated receptor-gamma agonist extends survival in transgenic mouse model of amyotrophic lateral sclerosis. Exp Neurol 191(2):331\u2013336","journal-title":"Exp Neurol"},{"issue":"2","key":"9286_CR34","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1001\/archneur.58.2.273","volume":"58","author":"SC Kirkwood","year":"2001","unstructured":"Kirkwood SC, Su JL, Conneally P, Foroud T (2001) Progression of symptoms in the early and middle stages of Huntington disease. Arch Neurol 58(2):273\u2013278","journal-title":"Arch Neurol"},{"issue":"2","key":"9286_CR35","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1016\/j.expneurol.2006.04.006","volume":"199","author":"G Landreth","year":"2006","unstructured":"Landreth G (2006) PPARgamma agonists as new therapeutic agents for the treatment of Alzheimer\u2019s disease. Exp Neurol 199(2):245\u2013248","journal-title":"Exp Neurol"},{"issue":"9","key":"9286_CR36","doi-asserted-by":"crossref","first-page":"5780","DOI":"10.1074\/jbc.M704704200","volume":"283","author":"D Lim","year":"2008","unstructured":"Lim D, Fedrizzi L, Tartari M, Zuccato C, Cattaneo E, Brini M, Carafoli E (2008) Calcium homeostasis and mitochondrial dysfunction in striatal neurons of Huntington disease. J Biol Chem 283(9):5780\u20135789","journal-title":"J Biol Chem"},{"issue":"1\u20132","key":"9286_CR37","first-page":"18","volume":"264","author":"CS Liu","year":"2008","unstructured":"Liu CS, Cheng WL, Kuo SJ, Li JY, Soong BW, Wei YH (2008) Depletion of mitochondrial DNA in leukocytes of patients with poly-Q diseases. J Neurol Sci 264(1\u20132):18\u201321","journal-title":"J Neurol Sci"},{"issue":"1","key":"9286_CR38","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1002\/1531-8249(200007)48:1<72::AID-ANA11>3.0.CO;2-I","volume":"48","author":"R Lodi","year":"2000","unstructured":"Lodi R, Schapira AH, Manners D, Styles P, Wood NW, Taylor DJ, Warner TT (2000) Abnormal in vivo skeletal muscle energy metabolism in Huntington\u2019s disease and dentatorubropallidoluysian atrophy. Ann Neurol 48(1):72\u201376","journal-title":"Ann Neurol"},{"issue":"2","key":"9286_CR39","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1111\/j.1471-4159.2006.03758.x","volume":"97","author":"Y Luo","year":"2006","unstructured":"Luo Y, Yin W, Signore AP, Zhang F, Hong Z, Wang S, Graham SH, Chen J (2006) Neuroprotection against focal ischemic brain injury by the peroxisome proliferator-activated receptor-gamma agonist rosiglitazone. J Neurochem 97(2):435\u2013448","journal-title":"J Neurochem"},{"issue":"3","key":"9286_CR40","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1016\/S0092-8674(00)81369-0","volume":"87","author":"L Mangiarini","year":"1996","unstructured":"Mangiarini L, Sathasivam K, Seller M, Cozens B, Harper A, Hetherington C, Lawton M, Trottier Y, Lehrach H, Davies SW et al (1996) Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 87(3):493\u2013506","journal-title":"Cell"},{"issue":"35","key":"9286_CR41","doi-asserted-by":"crossref","first-page":"30773","DOI":"10.1074\/jbc.M504749200","volume":"280","author":"T Milakovic","year":"2005","unstructured":"Milakovic T, Johnson GV (2005) Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin. J Biol Chem 280(35):30773\u201330782","journal-title":"J Biol Chem"},{"issue":"46","key":"9286_CR42","doi-asserted-by":"crossref","first-page":"34785","DOI":"10.1074\/jbc.M603845200","volume":"281","author":"T Milakovic","year":"2006","unstructured":"Milakovic T, Quintanilla RA, Johnson GV (2006) Mutant huntingtin expression induces mitochondrial calcium handling defects in clonal striatal cells: functional consequences. J Biol Chem 281(46):34785\u201334795","journal-title":"J Biol Chem"},{"issue":"1","key":"9286_CR43","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1016\/S0165-5728(01)00285-5","volume":"116","author":"M Niino","year":"2001","unstructured":"Niino M, Iwabuchi K, Kikuchi S, Ato M, Morohashi T, Ogata A, Tashiro K, Onoe K (2001) Amelioration of experimental autoimmune encephalomyelitis in C57BL\/6 mice by an agonist of peroxisome proliferator-activated receptor-gamma. J Neuroimmunol 116(1):40\u201348","journal-title":"J Neuroimmunol"},{"issue":"1","key":"9286_CR44","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1111\/j.1471-4159.2006.04361.x","volume":"101","author":"JM Oliveira","year":"2007","unstructured":"Oliveira JM, Jekabsons MB, Chen S, Lin A, Rego AC, Goncalves J, Ellerby LM, Nicholls DG (2007) Mitochondrial dysfunction in Huntington\u2019s disease: the bioenergetics of isolated and in situ mitochondria from transgenic mice. J Neurochem 101(1):241\u2013249","journal-title":"J Neurochem"},{"key":"9286_CR45","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1146\/annurev.neuro.29.051605.113042","volume":"30","author":"HT Orr","year":"2007","unstructured":"Orr HT, Zoghbi HY (2007) Trinucleotide repeat disorders. Annu Rev Neurosci 30:575\u2013621","journal-title":"Annu Rev Neurosci"},{"issue":"8","key":"9286_CR46","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1038\/nn884","volume":"5","author":"AV Panov","year":"2002","unstructured":"Panov AV, Gutekunst CA, Leavitt BR, Hayden MR, Burke JR, Strittmatter WJ, Greenamyre JT (2002) Early mitochondrial calcium defects in Huntington\u2019s disease are a direct effect of polyglutamines. Nat Neurosci 5(8):731\u2013736","journal-title":"Nat Neurosci"},{"issue":"4\u20135","key":"9286_CR47","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1016\/j.brainresbull.2009.07.010","volume":"80","author":"RA Quintanilla","year":"2009","unstructured":"Quintanilla RA, Johnson GV (2009) Role of mitochondrial dysfunction in the pathogenesis of Huntington\u2019s disease. Brain Res Bull 80(4\u20135):242\u2013247","journal-title":"Brain Res Bull"},{"issue":"37","key":"9286_CR48","doi-asserted-by":"crossref","first-page":"25628","DOI":"10.1074\/jbc.M804291200","volume":"283","author":"RA Quintanilla","year":"2008","unstructured":"Quintanilla RA, Jin YN, Fuenzalida K, Bronfman M, Johnson GV (2008) 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 283(37):25628\u201325637","journal-title":"J Biol Chem"},{"issue":"2","key":"9286_CR49","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1016\/0306-4522(96)00089-9","volume":"73","author":"M Rigby","year":"1996","unstructured":"Rigby M, Le Bourdelles B, Heavens RP, Kelly S, Smith D, Butler A, Hammans R, Hills R, Xuereb JH, Hill RG et al (1996) The messenger RNAs for the N-methyl-D-aspartate receptor subunits show region-specific expression of different subunit composition in the human brain. Neuroscience 73(2):429\u2013447","journal-title":"Neuroscience"},{"issue":"1","key":"9286_CR50","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.neuron.2007.11.025","volume":"57","author":"E Romero","year":"2008","unstructured":"Romero E, Cha GH, Verstreken P, Ly CV, Hughes RE, Bellen HJ, Botas J (2008) Suppression of neurodegeneration and increased neurotransmission caused by expanded full-length huntingtin accumulating in the cytoplasm. Neuron 57(1):27\u201340","journal-title":"Neuron"},{"issue":"11","key":"9286_CR51","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1038\/nm1106-1239","volume":"12","author":"CA Ross","year":"2006","unstructured":"Ross CA, Thompson LM (2006) Transcription meets metabolism in neurodegeneration. Nat Med 12(11):1239\u20131241","journal-title":"Nat Med"},{"issue":"8","key":"9286_CR52","doi-asserted-by":"crossref","first-page":"407","DOI":"10.5694\/j.1326-5377.1981.tb135681.x","volume":"1","author":"PR Sanberg","year":"1981","unstructured":"Sanberg PR, Fibiger HC, Mark RF (1981) Body weight and dietary factors in Huntington\u2019s disease patients compared with matched controls. Med J Aust 1(8):407\u2013409","journal-title":"Med J Aust"},{"issue":"1","key":"9286_CR53","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/S0092-8674(00)81782-1","volume":"95","author":"F Saudou","year":"1998","unstructured":"Saudou F, Finkbeiner S, Devys D, Greenberg ME (1998) Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 95(1):55\u201366","journal-title":"Cell"},{"issue":"3","key":"9286_CR54","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1016\/S0092-8674(00)80514-0","volume":"90","author":"E Scherzinger","year":"1997","unstructured":"Scherzinger E, Lurz R, Turmaine M, Mangiarini L, Hollenbach B, Hasenbank R, Bates GP, Davies SW, Lehrach H, Wanker EE (1997) Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell 90(3):549\u2013558","journal-title":"Cell"},{"key":"9286_CR55","doi-asserted-by":"crossref","first-page":"R115","DOI":"10.1093\/hmg\/ddm213","volume":"16","author":"J Shao","year":"2007","unstructured":"Shao J, Diamond MI (2007) Polyglutamine diseases: emerging concepts in pathogenesis and therapy. Hum Mol Genet 16:R115\u2013R123, Spec No 2","journal-title":"Hum Mol Genet"},{"issue":"1","key":"9286_CR56","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1038\/79139","volume":"26","author":"T Shimohata","year":"2000","unstructured":"Shimohata T, Nakajima T, Yamada M, Uchida C, Onodera O, Naruse S, Kimura T, Koide R, Nozaki K, Sano Y et al (2000) Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription. Nat Genet 26(1):29\u201336","journal-title":"Nat Genet"},{"issue":"8","key":"9286_CR57","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1016\/j.tig.2006.05.008","volume":"22","author":"EJ Slow","year":"2006","unstructured":"Slow EJ, Graham RK, Hayden MR (2006) To be or not to be toxic: aggregations in Huntington and Alzheimer disease. Trends Genet 22(8):408\u2013411","journal-title":"Trends Genet"},{"issue":"20","key":"9286_CR58","doi-asserted-by":"crossref","first-page":"3063","DOI":"10.1093\/hmg\/ddl248","volume":"15","author":"A Solans","year":"2006","unstructured":"Solans A, Zambrano A, Rodriguez M, Barrientos A (2006) Cytotoxicity of a mutant huntingtin fragment in yeast involves early alterations in mitochondrial OXPHOS complexes II and III. Hum Mol Genet 15(20):3063\u20133081","journal-title":"Hum Mol Genet"},{"issue":"1","key":"9286_CR59","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1002\/ana.410130116","volume":"13","author":"S Sorbi","year":"1983","unstructured":"Sorbi S, Bird ED, Blass JP (1983) Decreased pyruvate dehydrogenase complex activity in Huntington and Alzheimer brain. Ann Neurol 13(1):72\u201378","journal-title":"Ann Neurol"},{"issue":"2","key":"9286_CR60","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1016\/j.mad.2005.09.010","volume":"127","author":"F Squitieri","year":"2006","unstructured":"Squitieri F, Cannella M, Sgarbi G, Maglione V, Falleni A, Lenzi P, Baracca A, Cislaghi G, Saft C, Ragona G et al (2006) Severe ultrastructural mitochondrial changes in lymphoblasts homozygous for Huntington disease mutation. Mech Ageing Dev 127(2):217\u2013220","journal-title":"Mech Ageing Dev"},{"issue":"1","key":"9286_CR61","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1007\/s00702-009-0328-4","volume":"117","author":"F Squitieri","year":"2010","unstructured":"Squitieri F, Falleni A, Cannella M, Orobello S, Fulceri F, Lenzi P, Fornai F (2010) Abnormal morphology of peripheral cell tissues from patients with Huntington disease. J Neural Transm 117(1):77\u201383","journal-title":"J Neural Transm"},{"issue":"9","key":"9286_CR62","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1212\/WNL.24.9.813","volume":"24","author":"WL Stahl","year":"1974","unstructured":"Stahl WL, Swanson PD (1974) Biochemical abnormalities in Huntington\u2019s chorea brains. Neurology 24(9):813\u2013819","journal-title":"Neurology"},{"issue":"12","key":"9286_CR63","doi-asserted-by":"crossref","first-page":"6763","DOI":"10.1073\/pnas.100110097","volume":"97","author":"JS Steffan","year":"2000","unstructured":"Steffan JS, Kazantsev A, Spasic-Boskovic O, Greenwald M, Zhu YZ, Gohler H, Wanker EE, Bates GP, Housman DE, Thompson LM (2000) The Huntington\u2019s disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci U S A 97(12):6763\u20136768","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"6857","key":"9286_CR64","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1038\/35099568","volume":"413","author":"JS Steffan","year":"2001","unstructured":"Steffan JS, Bodai L, Pallos J, Poelman M, McCampbell A, Apostol BL, Kazantsev A, Schmidt E, Zhu YZ, Greenwald M et al (2001) Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila. Nature 413(6857):739\u2013743","journal-title":"Nature"},{"issue":"27","key":"9286_CR65","doi-asserted-by":"crossref","first-page":"24713","DOI":"10.1074\/jbc.M103501200","volume":"276","author":"Y Sun","year":"2001","unstructured":"Sun Y, Savanenin A, Reddy PH, Liu YF (2001) Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95. J Biol Chem 276(27):24713\u201324718","journal-title":"J Biol Chem"},{"issue":"3","key":"9286_CR66","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1016\/j.mcn.2005.07.016","volume":"30","author":"LA Swayne","year":"2005","unstructured":"Swayne LA, Chen L, Hameed S, Barr W, Charlesworth E, Colicos MA, Zamponi GW, Braun JE (2005) Crosstalk between huntingtin and syntaxin 1A regulates N-type calcium channels. Mol Cell Neurosci 30(3):339\u2013351","journal-title":"Mol Cell Neurosci"},{"issue":"1","key":"9286_CR67","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1002\/1531-8249(200001)47:1<80::AID-ANA13>3.0.CO;2-K","volume":"47","author":"SJ Tabrizi","year":"2000","unstructured":"Tabrizi SJ, Workman J, Hart PE, Mangiarini L, Mahal A, Bates G, Cooper JM, Schapira AH (2000) Mitochondrial dysfunction and free radical damage in the Huntington R6\/2 transgenic mouse. Ann Neurol 47(1):80\u201386","journal-title":"Ann Neurol"},{"issue":"2","key":"9286_CR68","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1016\/S0896-6273(03)00366-0","volume":"39","author":"TS Tang","year":"2003","unstructured":"Tang TS, Tu H, Chan EY, Maximov A, Wang Z, Wellington CL, Hayden MR, Bezprozvanny I (2003) Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1, 4, 5) triphosphate receptor type 1. Neuron 39(2):227\u2013239","journal-title":"Neuron"},{"issue":"7","key":"9286_CR69","doi-asserted-by":"crossref","first-page":"2602","DOI":"10.1073\/pnas.0409402102","volume":"102","author":"TS Tang","year":"2005","unstructured":"Tang TS, Slow E, Lupu V, Stavrovskaya IG, Sugimori M, Llinas R, Kristal BS, Hayden MR, Bezprozvanny I (2005) Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington\u2019s disease. Proc Natl Acad Sci U S A 102(7):2602\u20132607","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"2","key":"9286_CR70","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1097\/00005072-197404000-00008","volume":"33","author":"I Tellez-Nagel","year":"1974","unstructured":"Tellez-Nagel I, Johnson AB, Terry RD (1974) Studies on brain biopsies of patients with Huntington\u2019s chorea. J Neuropathol Exp Neurol 33(2):308\u2013332","journal-title":"J Neuropathol Exp Neurol"},{"issue":"6","key":"9286_CR71","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","volume":"72","author":"The Huntington\u2019s Disease Collaborative Research Group","year":"1993","unstructured":"The Huntington\u2019s Disease Collaborative Research Group (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington\u2019s disease chromosomes. Cell 72(6):971\u2013983","journal-title":"Cell"},{"issue":"19","key":"9286_CR72","doi-asserted-by":"crossref","first-page":"2799","DOI":"10.1093\/hmg\/9.19.2799","volume":"9","author":"F Trettel","year":"2000","unstructured":"Trettel F, Rigamonti D, Hilditch-Maguire P, Wheeler VC, Sharp AH, Persichetti F, Cattaneo E, MacDonald ME (2000) Dominant phenotypes produced by the HD mutation in STHdh(Q111) striatal cells. Hum Mol Genet 9(19):2799\u20132809","journal-title":"Hum Mol Genet"},{"issue":"12","key":"9286_CR73","doi-asserted-by":"crossref","first-page":"1715","DOI":"10.1002\/mds.21540","volume":"22","author":"C Turner","year":"2007","unstructured":"Turner C, Cooper JM, Schapira AH (2007) Clinical correlates of mitochondrial function in Huntington\u2019s disease muscle. Mov Disord 22(12):1715\u20131721","journal-title":"Mov Disord"},{"issue":"6","key":"9286_CR74","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1016\/S0896-6273(02)00615-3","volume":"33","author":"MM Zeron","year":"2002","unstructured":"Zeron MM, Hansson O, Chen N, Wellington CL, Leavitt BR, Brundin P, Hayden MR, Raymond LA (2002) Increased sensitivity to N-methyl-D-aspartate receptor-mediated excitotoxicity in a mouse model of Huntington\u2019s disease. Neuron 33(6):849\u2013860","journal-title":"Neuron"}],"container-title":["Journal of Bioenergetics and Biomembranes"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10863-010-9286-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s10863-010-9286-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10863-010-9286-7","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,6,5]],"date-time":"2020-06-05T20:03:20Z","timestamp":1591387400000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s10863-010-9286-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,6]]},"references-count":74,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2010,6]]}},"alternative-id":["9286"],"URL":"https:\/\/doi.org\/10.1007\/s10863-010-9286-7","relation":{},"ISSN":["0145-479X","1573-6881"],"issn-type":[{"value":"0145-479X","type":"print"},{"value":"1573-6881","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,6]]}}}