{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T23:49:02Z","timestamp":1792540142317,"version":"4.5.0"},"reference-count":132,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2017,6,21]],"date-time":"2017-06-21T00:00:00Z","timestamp":1498003200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Neurotox Res"],"published-print":{"date-parts":[[2017,10]]},"DOI":"10.1007\/s12640-017-9766-1","type":"journal-article","created":{"date-parts":[[2017,6,21]],"date-time":"2017-06-21T06:51:53Z","timestamp":1498027913000},"page":"518-529","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":141,"title":["Huntington\u2019s Disease and Mitochondria"],"prefix":"10.1007","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5254-6572","authenticated-orcid":false,"given":"Mohammad","family":"Jodeiri Farshbaf","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kamran","family":"Ghaedi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2017,6,21]]},"reference":[{"key":"9766_CR1","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1038\/ng1095-115","volume":"11","author":"MA Andrade","year":"1995","unstructured":"Andrade MA, Bork P (1995) HEAT repeats in the Huntington\u2019s disease protein. Nat Genet 11:115\u2013116","journal-title":"Nat Genet"},{"key":"9766_CR2","doi-asserted-by":"crossref","first-page":"44735","DOI":"10.1074\/jbc.M302673200","volume":"278","author":"MA Aon","year":"2003","unstructured":"Aon MA, Cortassa S, Marb\u00e1n E, O\u2019Rourke B (2003) Synchronized whole cell oscillations in mitochondrial metabolism triggered by a local release of reactive oxygen species in cardiac myocytes. J Biol Chem 278:44735\u201344744","journal-title":"J Biol Chem"},{"key":"9766_CR3","doi-asserted-by":"crossref","first-page":"1010","DOI":"10.1126\/science.1098014","volume":"305","author":"T Araki","year":"2004","unstructured":"Araki T, Sasaki Y, Milbrandt J (2004) Increased nuclear NAD biosynthesis and SIRT1 activation prevent axonal degeneration. Science 305:1010\u20131013","journal-title":"Science"},{"key":"9766_CR4","first-page":"187","volume":"11","author":"G Ashrafi","year":"2015","unstructured":"Ashrafi G, Schwarz TL (2015) PINK1- and PARK2-mediated local mitophagy in distal neuronal axons. Autophagy 11:187\u2013189","journal-title":"Autophagy"},{"key":"9766_CR5","doi-asserted-by":"crossref","first-page":"360438","DOI":"10.1155\/2014\/360438","volume":"2014","author":"A Ayala","year":"2014","unstructured":"Ayala A, Mu\u00f1oz MF, Arg\u00fcelles S (2014) Lipid peroxidation: production, metabolism, and signaling mechanisms of malondialdehyde and 4-hydroxy-2-nonenal. Oxidative Med Cell Longev 2014:360438","journal-title":"Oxidative Med Cell Longev"},{"issue":"1","key":"9766_CR6","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/j.neuron.2005.06.005","volume":"47","author":"BI Bae","year":"2005","unstructured":"Bae BI, Xu H, Igarashi S, Fujimuro M, Agrawal N, Taya Y, Hayward SD, Moran TH, Montell C, Ross CA, Snyder SH, Sawa A (2005) p53 mediates cellular dysfunction and behavioral abnormalities in Huntington\u2019s disease. Neuron 47(1):29\u201341","journal-title":"Neuron"},{"key":"9766_CR7","doi-asserted-by":"crossref","unstructured":"Bennett EJ, Shaler TA, Woodman B, Ryu KY, Zaitseva TS, Becker CH, Bates GP, Schulman H, Kopito RR (2007) Global changes to the ubiquitin system in Huntington's disease. Nature. 9;448(7154):704\u20138","DOI":"10.1038\/nature06022"},{"key":"9766_CR8","doi-asserted-by":"crossref","first-page":"1127","DOI":"10.1152\/physrev.1999.79.4.1127","volume":"79","author":"P Bernardi","year":"1999","unstructured":"Bernardi P (1999) Mitochondrial transport of cations: channels, exchangers, and permeability transition. Physiol Rev 79:1127\u20131155","journal-title":"Physiol Rev"},{"key":"9766_CR9","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1093\/hmg\/ddi423","volume":"15","author":"JS Bett","year":"2006","unstructured":"Bett JS, Goellner GM, Woodman B, Pratt G, Rechsteiner M, Bates GP (2006) Proteasome impairment does not contribute to pathogenesis in R6\/2 Huntington\u2019s disease mice: exclusion of proteasome activator REGgamma as a therapeutic target. Hum Mol Genet 15:33\u201344","journal-title":"Hum Mol Genet"},{"key":"9766_CR10","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1016\/j.tins.2008.09.004","volume":"31","author":"E Bossy-Wetzel","year":"2008","unstructured":"Bossy-Wetzel E, Petrilli A, Knott AB (2008) Mutant huntingtin and mitochondrial dysfunction. Trends Neurosci 31:609\u2013616","journal-title":"Trends Neurosci"},{"key":"9766_CR11","doi-asserted-by":"crossref","first-page":"C817","DOI":"10.1152\/ajpcell.00139.2004","volume":"287","author":"PS Brookes","year":"2004","unstructured":"Brookes PS, Yoon Y, Robotham JL, Anders MW, Sheu SS (2004) Calcium, ATP, and ROS: a mitochondrial love-hate triangle. Am J Physiol Cell Physiol 287:C817\u2013C833","journal-title":"Am J Physiol Cell Physiol"},{"key":"9766_CR12","doi-asserted-by":"crossref","first-page":"2944","DOI":"10.1007\/s12035-015-9188-0","volume":"53","author":"N Brustovetsky","year":"2016","unstructured":"Brustovetsky N (2016) Mutant huntingtin and elusive defects in oxidative metabolism and mitochondrial calcium handling. Mol Neurobiol 53:2944\u20132953","journal-title":"Mol Neurobiol"},{"key":"9766_CR13","doi-asserted-by":"crossref","first-page":"14610","DOI":"10.1073\/pnas.1301342110","volume":"110","author":"NS Caron","year":"2013","unstructured":"Caron NS, Desmond CR, Xia J, Truant R (2013) Polyglutamine domain flexibility mediates the proximity between flanking sequences in huntingtin. Proc Natl Acad Sci U S A 110:14610\u201314615","journal-title":"Proc Natl Acad Sci U S A"},{"key":"9766_CR14","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/j.tcb.2009.01.005","volume":"19","author":"JP Caviston","year":"2009","unstructured":"Caviston JP, Holzbaur EL (2009) Huntingtin as an essential integrator of intracellular vesicular trafficking. Trends Cell Biol 19:147\u2013155","journal-title":"Trends Cell Biol"},{"key":"9766_CR15","doi-asserted-by":"crossref","first-page":"1241","DOI":"10.1016\/j.cell.2006.06.010","volume":"125","author":"DC Chan","year":"2006","unstructured":"Chan DC (2006) Mitochondria: dynamic organelles in disease, aging, and development. Cell 125:1241\u20131252","journal-title":"Cell"},{"key":"9766_CR16","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1111\/j.1749-6632.2010.05629.x","volume":"1201","author":"CR Chang","year":"2010","unstructured":"Chang CR, Blackstone C (2010) Dynamic regulation of mitochondrial fission through modification of the dynamin-related protein Drp1. Ann N Y Acad Sci 1201:34\u201339","journal-title":"Ann N Y Acad Sci"},{"key":"9766_CR17","doi-asserted-by":"crossref","first-page":"7391","DOI":"10.1021\/bi011772q","volume":"41","author":"S Chen","year":"2002","unstructured":"Chen S, Berthelier V, Hamilton JB, O\u2019Nuallain B, Wetzel R (2002) Amyloid-like features of polyglutamine aggregates and their assembly kinetics. Biochemistry 41:7391\u20137399","journal-title":"Biochemistry"},{"key":"9766_CR18","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 13:1407\u20131420","journal-title":"Hum Mol Genet"},{"key":"9766_CR19","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1111\/j.1471-4159.2005.03029.x","volume":"93","author":"SY Chou","year":"2005","unstructured":"Chou SY, Lee YC, Chen HM, Chiang MC, Lai HL, Chang HH, Wu YC, Sun CN, Chien CL, Lin YS, Wang SC, Tung YY, Chang C, Chern Y (2005) CGS21680 attenuates symptoms of Huntington\u2019s disease in a transgenic mouse model. J Neurochem 93:310\u2013320","journal-title":"J Neurochem"},{"key":"9766_CR20","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1038\/sj.embor.7401062","volume":"8","author":"JT Cribbs","year":"2007","unstructured":"Cribbs JT, Strack S (2007) Reversible phosphorylation of Drp1 by cyclic AMP-dependent protein kinase and calcineurin regulates mitochondrial fission and cell death. EMBO Rep 8:939\u2013944","journal-title":"EMBO Rep"},{"issue":"Pt 2","key":"9766_CR21","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1042\/bj3410233","volume":"341","author":"M Crompton","year":"1999","unstructured":"Crompton M (1999) The mitochondrial permeability transition pore and its role in cell death. Biochem J 341(Pt 2):233\u2013249","journal-title":"Biochem J"},{"key":"9766_CR22","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 127:59\u201369","journal-title":"Cell"},{"key":"9766_CR23","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1093\/cvr\/24.5.411","volume":"24","author":"AM Das","year":"1990","unstructured":"Das AM, Harris DA (1990) Control of mitochondrial ATP synthase in heart cells: inactive to active transitions caused by beating or positive inotropic agents. Cardiovasc Res 24:411\u2013417","journal-title":"Cardiovasc Res"},{"key":"9766_CR24","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1038\/79936","volume":"26","author":"C Delettre","year":"2000","unstructured":"Delettre C, Lenaers G, Griffoin JM, Gigarel N, Lorenzo C, Belenguer P, Pelloquin L, Grosgeorge J, Turc-Carel C, Perret E, Astarie-Dequeker C, Lasquellec L, Arnaud B, Ducommun B, Kaplan J, Hamel CP (2000) Nuclear gene OPA1, encoding a mitochondrial dynamin-related protein, is mutated in dominant optic atrophy. Nat Genet 26:207\u2013210","journal-title":"Nat Genet"},{"key":"9766_CR25","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 277:1990\u20131993","journal-title":"Science"},{"key":"9766_CR26","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1096\/fj.00-0262com","volume":"15","author":"RJ Donahue","year":"2001","unstructured":"Donahue RJ, Razmara M, Hoek JB, Knudsen TB (2001) Direct influence of the p53 tumor suppressor on mitochondrial biogenesis and function. FASEB J 15:635\u2013644","journal-title":"FASEB J"},{"key":"9766_CR27","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1038\/81593","volume":"26","author":"I Dragatsis","year":"2000","unstructured":"Dragatsis I, Levine MS, Zeitlin S (2000) Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice. Nat Genet 26:300\u2013306","journal-title":"Nat Genet"},{"key":"9766_CR28","doi-asserted-by":"crossref","first-page":"2255","DOI":"10.15252\/embj.201490808","volume":"34","author":"MT El-Daher","year":"2015","unstructured":"El-Daher MT, Hangen E, Bruy\u00e8re J, Poizat G, Al-Ramahi I, Pardo R, Bourg N, Souquere S, Mayet C, Pierron G, L\u00e9v\u00eaque-Fort S, Botas J, Humbert S, Saudou F (2015) Huntingtin proteolysis releases non-polyQ fragments that cause toxicity through dynamin 1 dysregulation. EMBO J 34:2255\u20132271","journal-title":"EMBO J"},{"key":"9766_CR29","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1016\/j.bbamcr.2012.05.002","volume":"1833","author":"K Elgass","year":"2013","unstructured":"Elgass K, Pakay J, Ryan MT, Palmer CS (2013) Recent advances into the understanding of mitochondrial fission. Biochim Biophys Acta 1833:150\u2013161","journal-title":"Biochim Biophys Acta"},{"key":"9766_CR30","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1016\/j.bbamcr.2012.07.016","volume":"1833","author":"M Escobar-Henriques","year":"2013","unstructured":"Escobar-Henriques M, Anton F (2013) Mechanistic perspective of mitochondrial fusion: tubulation vs. fragmentation. Biochim Biophys Acta 1833:162\u2013175","journal-title":"Biochim Biophys Acta"},{"key":"9766_CR31","doi-asserted-by":"crossref","first-page":"1086","DOI":"10.1038\/sj.cdd.4402107","volume":"14","author":"J Estaquier","year":"2007","unstructured":"Estaquier J, Arnoult D (2007) Inhibiting Drp1-mediated mitochondrial fission selectively prevents the release of cytochrome c during apoptosis. Cell Death Differ 14:1086\u20131094","journal-title":"Cell Death Differ"},{"key":"9766_CR32","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/sj.onc.1209551","volume":"25","author":"Z Feng","year":"2006","unstructured":"Feng Z, Jin S, Zupnick A, Hoh J, de Stanchina E, Lowe S, Prives C, Levine AJ (2006) p53 tumor suppressor protein regulates the levels of huntingtin gene expression. Oncogene 25:1\u20137","journal-title":"Oncogene"},{"key":"9766_CR33","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1016\/j.tibs.2012.02.004","volume":"37","author":"F Fischer","year":"2012","unstructured":"Fischer F, Hamann A, Osiewacz HD (2012) Mitochondrial quality control: an integrated network of pathways. Trends Biochem Sci 37:284\u2013292","journal-title":"Trends Biochem Sci"},{"key":"9766_CR34","doi-asserted-by":"crossref","unstructured":"Font\u00e1n-Lozano A, L\u00f3pez-Lluch G, Delgado-Garc\u00eda JM, Navas P, Carri\u00f3n AM (2008) Molecular bases of caloric restriction regulation of neuronal synaptic plasticity. Mol Neurobiol. 38(2):167\u201377","DOI":"10.1007\/s12035-008-8040-1"},{"key":"9766_CR35","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.cell.2004.06.018","volume":"118","author":"LR Gauthier","year":"2004","unstructured":"Gauthier LR, Charrin BC, Borrell-Pag\u00e8s M, Dompierre JP, Rangone H, Cordeli\u00e8res FP, De Mey J, MacDonald ME, Lessmann V, Humbert S, Saudou F (2004) Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules. Cell 118:127\u2013138","journal-title":"Cell"},{"key":"9766_CR36","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1042\/bj3580147","volume":"358","author":"D Gincel","year":"2001","unstructured":"Gincel D, Zaid H, Shoshan-Barmatz V (2001) Calcium binding and translocation by the voltage-dependent anion channel: a possible regulatory mechanism in mitochondrial function. Biochem J 358:147\u2013155","journal-title":"Biochem J"},{"key":"9766_CR37","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1038\/ncb2220","volume":"13","author":"LC Gomes","year":"2011","unstructured":"Gomes LC, Di Benedetto G, Scorrano L (2011) During autophagy mitochondria elongate, are spared from degradation and sustain cell viability. Nat Cell Biol 13:589\u2013598","journal-title":"Nat Cell Biol"},{"key":"9766_CR38","doi-asserted-by":"crossref","first-page":"1077","DOI":"10.1023\/A:1026398310003","volume":"68","author":"AV Gordeeva","year":"2003","unstructured":"Gordeeva AV, Zvyagilskaya RA, Labas YA (2003) Cross-talk between reactive oxygen species and calcium in living cells. Biochemistry (Mosc) 68:1077\u20131080","journal-title":"Biochemistry (Mosc)"},{"key":"9766_CR39","doi-asserted-by":"crossref","unstructured":"Greenberg ME, Xu B, Lu B, Hempstead BL (2009) New insights in the biology of BDNF synthesis and release: implications in CNS function. J Neurosci. 14;29(41):12764\u20137","DOI":"10.1523\/JNEUROSCI.3566-09.2009"},{"key":"9766_CR40","doi-asserted-by":"crossref","first-page":"2484","DOI":"10.1016\/j.bbadis.2015.08.012","volume":"1852","author":"P Guedes-Dias","year":"2015","unstructured":"Guedes-Dias P, de Proen\u00e7a J, Soares TR, Leit\u00e3o-Rocha A, Pinho BR, Duchen MR, Oliveira JM (2015) HDAC6 inhibition induces mitochondrial fusion, autophagic flux and reduces diffuse mutant huntingtin in striatal neurons. Biochim Biophys Acta 1852:2484\u20132493","journal-title":"Biochim Biophys Acta"},{"key":"9766_CR41","doi-asserted-by":"crossref","first-page":"C313","DOI":"10.1152\/ajpcell.1994.267.2.C313","volume":"267","author":"TE Gunter","year":"1994","unstructured":"Gunter TE, Gunter KK, Sheu SS, Gavin CE (1994) Mitochondrial calcium transport: physiological and pathological relevance. Am J Phys 267:C313\u2013C339","journal-title":"Am J Phys"},{"key":"9766_CR42","doi-asserted-by":"crossref","first-page":"7674","DOI":"10.1523\/JNEUROSCI.18-19-07674.1998","volume":"18","author":"CA Gutekunst","year":"1998","unstructured":"Gutekunst CA, Li SH, Yi H, Ferrante RJ, Li XJ, Hersch SM (1998) The cellular and subcellular localization of huntingtin-associated protein 1 (HAP1): comparison with huntingtin in rat and human. J Neurosci 18:7674\u20137686","journal-title":"J Neurosci"},{"key":"9766_CR43","doi-asserted-by":"crossref","first-page":"1895","DOI":"10.1101\/gad.17420111","volume":"25","author":"DG Hardie","year":"2011","unstructured":"Hardie DG (2011) AMP-activated protein kinase: an energy sensor that regulates all aspects of cell function. Genes Dev 25:1895\u20131908","journal-title":"Genes Dev"},{"key":"9766_CR44","doi-asserted-by":"crossref","first-page":"1387","DOI":"10.1007\/s00415-014-7350-7","volume":"261","author":"M Herben-Dekker","year":"2014","unstructured":"Herben-Dekker M, van Oostrom JC, Roos RA, Jurgens CK, Witjes-An\u00e9 MN, Kremer HP, Leenders KL, Spikman JM (2014) Striatal metabolism and psychomotor speed as predictors of motor onset in Huntington\u2019s disease. J Neurol 261:1387\u20131397","journal-title":"J Neurol"},{"key":"9766_CR45","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/S0896-6273(00)80764-3","volume":"23","author":"JG Hodgson","year":"1999","unstructured":"Hodgson JG, Agopyan N, Gutekunst CA, Leavitt BR, LePiane F, Singaraja R, Smith DJ, Bissada N, McCutcheon K, Nasir J, Jamot L, Li XJ, Stevens ME, Rosemond E, Roder JC, Phillips AG, Rubin EM, Hersch SM, Hayden MR (1999) A YAC mouse model for Huntington\u2019s disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. Neuron 23:181\u2013192","journal-title":"Neuron"},{"key":"9766_CR46","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1016\/j.ydbio.2014.04.005","volume":"391","author":"I Ismailoglu","year":"2014","unstructured":"Ismailoglu I, Chen Q, Popowski M, Yang L, Gross SS, Brivanlou AH (2014) Huntingtin protein is essential for mitochondrial metabolism, bioenergetics and structure in murine embryonic stem cells. Dev Biol 391:230\u2013240","journal-title":"Dev Biol"},{"key":"9766_CR47","doi-asserted-by":"crossref","unstructured":"Jacobsen JC, Gregory GC, Woda JM, Thompson MN, Coser KR, Murthy V, Kohane IS, Gusella JF, Seong IS, MacDonald ME, Shioda T, Lee JM (2011) HD CAG-correlated gene expression changes support a simple dominant gain of function. Hum Mol Genet. 20(14):2846\u201360","DOI":"10.1093\/hmg\/ddr195"},{"key":"9766_CR48","doi-asserted-by":"crossref","first-page":"23788","DOI":"10.1074\/jbc.M703812200","volume":"282","author":"A Jahani-Asl","year":"2007","unstructured":"Jahani-Asl A, Cheung EC, Neuspiel M, MacLaurin JG, Fortin A, Park DS, McBride HM, Slack RS (2007) Mitofusin 2 protects cerebellar granule neurons against injury-induced cell death. J Biol Chem 282:23788\u201323798","journal-title":"J Biol Chem"},{"key":"9766_CR49","doi-asserted-by":"crossref","first-page":"574","DOI":"10.1111\/j.1600-0854.2008.00701.x","volume":"9","author":"L Jahreiss","year":"2008","unstructured":"Jahreiss L, Menzies FM, Rubinsztein DC (2008) The itinerary of autophagosomes: from peripheral formation to kiss-and-run fusion with lysosomes. Traffic 9:574\u2013587","journal-title":"Traffic"},{"key":"9766_CR50","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1038\/nm.2558","volume":"18","author":"M Jiang","year":"2011","unstructured":"Jiang M, Wang J, Fu J, Du L, Jeong H, West T, Xiang L, Peng Q, Hou Z, Cai H, Seredenina T, Arbez N, Zhu S, Sommers K, Qian J, Zhang J, Mori S, Yang XW, Tamashiro KL, Aja S et al (2011) Neuroprotective role of Sirt1 in mammalian models of Huntington\u2019s disease through activation of multiple Sirt1 targets. Nat Med 18:153\u2013158","journal-title":"Nat Med"},{"key":"9766_CR51","doi-asserted-by":"crossref","first-page":"4139","DOI":"10.1242\/jcs.114.23.4139","volume":"114","author":"S Jin","year":"2001","unstructured":"Jin S, Levine AJ (2001) The p53 functional circuit. J Cell Sci 114:4139\u20134140","journal-title":"J Cell Sci"},{"key":"9766_CR52","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1083\/jcb.201105010","volume":"194","author":"TC Ju","year":"2011","unstructured":"Ju TC, Chen HM, Lin JT, Chang CP, Chang WC, Kang JJ, Sun CP, Tao MH, Tu PH, Chang C, Dickson DW, Chern Y (2011) Nuclear translocation of AMPK-alpha1 potentiates striatal neurodegeneration in Huntington\u2019s disease. J Cell Biol 194:209\u2013227","journal-title":"J Cell Biol"},{"key":"9766_CR53","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1083\/jcb.201110034","volume":"197","author":"Y Kageyama","year":"2012","unstructured":"Kageyama Y, Zhang Z, Roda R, Fukaya M, Wakabayashi J, Wakabayashi N, Kensler TW, Reddy PH, Iijima M, Sesaki H (2012) Mitochondrial division ensures the survival of postmitotic neurons by suppressing oxidative damage. J Cell Biol 197:535\u2013551","journal-title":"J Cell Biol"},{"key":"9766_CR54","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1196\/annals.1338.010","volume":"1042","author":"D Kang","year":"2005","unstructured":"Kang D, Hamasaki N (2005) Mitochondrial transcription factor A in the maintenance of mitochondrial DNA: overview of its multiple roles. Ann N Y Acad Sci 1042:101\u2013108","journal-title":"Ann N Y Acad Sci"},{"key":"9766_CR55","doi-asserted-by":"crossref","first-page":"7268","DOI":"10.1523\/JNEUROSCI.20-19-07268.2000","volume":"20","author":"KB Kegel","year":"2000","unstructured":"Kegel KB, Kim M, Sapp E, McIntyre C, Casta\u00f1o JG, Aronin N, DiFiglia M (2000) Huntingtin expression stimulates endosomal-lysosomal activity, endosome tubulation, and autophagy. J Neurosci 20:7268\u20137278","journal-title":"J Neurosci"},{"key":"9766_CR56","doi-asserted-by":"crossref","first-page":"e1617","DOI":"10.1038\/cddis.2014.581","volume":"6","author":"B Khalil","year":"2015","unstructured":"Khalil B, El Fissi N, Aouane A, Cabirol-Pol MJ, Rival T, Li\u00e9vens JC (2015) PINK1-induced mitophagy promotes neuroprotection in Huntington\u2019s disease. Cell Death Dis 6:e1617","journal-title":"Cell Death Dis"},{"key":"9766_CR57","doi-asserted-by":"crossref","first-page":"3919","DOI":"10.1093\/hmg\/ddq306","volume":"19","author":"J Kim","year":"2010","unstructured":"Kim J, Moody JP, Edgerly CK, Bordiuk OL, Cormier K, Smith K, Beal MF, Ferrante RJ (2010) Mitochondrial loss, dysfunction and altered dynamics in Huntington\u2019s disease. Hum Mol Genet 19:3919\u20133935","journal-title":"Hum Mol Genet"},{"key":"9766_CR58","doi-asserted-by":"crossref","first-page":"1109","DOI":"10.1016\/j.cell.2006.11.013","volume":"127","author":"M Lagouge","year":"2006","unstructured":"Lagouge M, Argmann C, Gerhart-Hines Z, Meziane H, Lerin C, Daussin F, Messadeq N, Milne J, Lambert P, Elliott P, Geny B, Laakso M, Puigserver P, Auwerx J (2006) Resveratrol improves mitochondrial function and protects against metabolic disease by activating SIRT1 and PGC-1alpha. Cell 127:1109\u20131122","journal-title":"Cell"},{"key":"9766_CR59","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1111\/j.1399-0004.2004.00241.x","volume":"65","author":"DR Langbehn","year":"2004","unstructured":"Langbehn DR, Brinkman RR, Falush D, Paulsen JS, Hayden MR, Group IHsDC (2004) A new model for prediction of the age of onset and penetrance for Huntington\u2019s disease based on CAG length. Clin Genet 65:267\u2013277","journal-title":"Clin Genet"},{"key":"9766_CR60","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1146\/annurev.ge.29.120195.001055","volume":"29","author":"NG Larsson","year":"1995","unstructured":"Larsson NG, Clayton DA (1995) Molecular genetic aspects of human mitochondrial disorders. Annu Rev Genet 29:151\u2013178","journal-title":"Annu Rev Genet"},{"key":"9766_CR61","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1086\/318207","volume":"68","author":"BR Leavitt","year":"2001","unstructured":"Leavitt BR, Guttman JA, Hodgson JG, Kimel GH, Singaraja R, Vogl AW, Hayden MR (2001) Wild-type huntingtin reduces the cellular toxicity of mutant huntingtin in vivo. Am J Hum Genet 68:313\u2013324","journal-title":"Am J Hum Genet"},{"key":"9766_CR62","doi-asserted-by":"crossref","unstructured":"Li SH, Li XJ (2004) Huntingtin and its role in neuronal degeneration. Neuroscientist. 10(5):467\u201375","DOI":"10.1177\/1073858404266777"},{"key":"9766_CR63","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.cell.2004.09.013","volume":"119","author":"J Lin","year":"2004","unstructured":"Lin J, Wu PH, Tarr PT, Lindenberg KS, St-Pierre J, Zhang CY, Mootha VK, J\u00e4ger S, Vianna CR, Reznick RM, Cui L, Manieri M, Donovan MX, Wu Z, Cooper MP, Fan MC, Rohas LM, Zavacki AM, Cinti S, Shulman GI et al (2004b) Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice. Cell 119:121\u2013135","journal-title":"Cell"},{"key":"9766_CR64","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1038\/nature05292","volume":"443","author":"MT Lin","year":"2006","unstructured":"Lin MT, Beal MF (2006) Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases. Nature 443:787\u2013795","journal-title":"Nature"},{"key":"9766_CR65","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1101\/gad.1164804","volume":"18","author":"SJ Lin","year":"2004","unstructured":"Lin SJ, Ford E, Haigis M, Liszt G, Guarente L (2004a) Calorie restriction extends yeast life span by lowering the level of NADH. Genes Dev 18:12\u201316","journal-title":"Genes Dev"},{"key":"9766_CR66","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1016\/j.neulet.2006.10.039","volume":"411","author":"TC Ma","year":"2007","unstructured":"Ma TC, Buescher JL, Oatis B, Funk JA, Nash AJ, Carrier RL, Hoyt KR (2007) Metformin therapy in a transgenic mouse model of Huntington\u2019s disease. Neurosci Lett 411:98\u2013103","journal-title":"Neurosci Lett"},{"issue":"1","key":"9766_CR67","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1634\/stemcells.2007-0221","volume":"26","author":"L Madhavan","year":"2008","unstructured":"Madhavan L, Ourednik V, Ourednik J (2008) Neural stem\/progenitor cells initiate the formation of cellular networks that provide neuroprotection by growth factor-modulated antioxidant expression. Stem Cells 26(1):254\u2013265","journal-title":"Stem Cells"},{"key":"9766_CR68","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1016\/j.redox.2015.02.001","volume":"4","author":"RJ Mailloux","year":"2015","unstructured":"Mailloux RJ (2015) Teaching the fundamentals of electron transfer reactions in mitochondria and the production and detection of reactive oxygen species. Redox Biol 4:381\u2013398","journal-title":"Redox Biol"},{"key":"9766_CR69","doi-asserted-by":"crossref","first-page":"1189","DOI":"10.1111\/j.1460-9568.2004.03578.x","volume":"20","author":"A Markham","year":"2004","unstructured":"Markham A, Cameron I, Franklin P, Spedding M (2004) BDNF increases rat brain mitochondrial respiratory coupling at complex I, but not complex II. Eur J Neurosci 20:1189\u20131196","journal-title":"Eur J Neurosci"},{"key":"9766_CR70","doi-asserted-by":"crossref","first-page":"2422","DOI":"10.1093\/hmg\/ddr148","volume":"20","author":"E Martin","year":"2011","unstructured":"Martin E, Betuing S, Pag\u00e8s C, Cambon K, Auregan G, Deglon N, Roze E, Caboche J (2011) Mitogen- and stress-activated protein kinase 1-induced neuroprotection in Huntington\u2019s disease: role on chromatin remodeling at the PGC-1-alpha promoter. Hum Mol Genet 20:2422\u20132434","journal-title":"Hum Mol Genet"},{"key":"9766_CR71","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1016\/B978-0-12-385883-2.00005-9","volume":"107","author":"LJ Martin","year":"2012","unstructured":"Martin LJ (2012) Biology of mitochondria in neurodegenerative diseases. Prog Mol Biol Transl Sci 107:355\u2013415","journal-title":"Prog Mol Biol Transl Sci"},{"key":"9766_CR72","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1038\/nn.2528","volume":"13","author":"M Martinez-Vicente","year":"2010","unstructured":"Martinez-Vicente M, Talloczy Z, Wong E, Tang G, Koga H, Kaushik S, de Vries R, Arias E, Harris S, Sulzer D, Cuervo AM (2010) Cargo recognition failure is responsible for inefficient autophagy in Huntington\u2019s disease. Nat Neurosci 13:567\u2013576","journal-title":"Nat Neurosci"},{"key":"9766_CR73","doi-asserted-by":"crossref","first-page":"748","DOI":"10.1016\/j.neuron.2008.10.010","volume":"60","author":"MP Mattson","year":"2008","unstructured":"Mattson MP, Gleichmann M, Cheng A (2008) Mitochondria in neuroplasticity and neurological disorders. Neuron 60:748\u2013766","journal-title":"Neuron"},{"key":"9766_CR74","doi-asserted-by":"crossref","first-page":"2467","DOI":"10.2337\/db06-1465","volume":"56","author":"A Mazzucotelli","year":"2007","unstructured":"Mazzucotelli A, Viguerie N, Tiraby C, Annicotte JS, Mairal A, Klimcakova E, Lepin E, Delmar P, Dejean S, Tavernier G, Lefort C, Hidalgo J, Pineau T, Fajas L, Cl\u00e9ment K, Langin D (2007) The transcriptional coactivator peroxisome proliferator activated receptor (PPAR)gamma coactivator-1 alpha and the nuclear receptor PPAR alpha control the expression of glycerol kinase and metabolism genes independently of PPAR gamma activation in human white adipocytes. Diabetes 56:2467\u20132475","journal-title":"Diabetes"},{"key":"9766_CR75","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1159\/000111332","volume":"15","author":"JG McCormack","year":"1993","unstructured":"McCormack JG, Denton RM (1993) Mitochondrial Ca2+ transport and the role of intramitochondrial Ca2+ in the regulation of energy metabolism. Dev Neurosci 15:165\u2013173","journal-title":"Dev Neurosci"},{"key":"9766_CR76","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1016\/j.cell.2006.10.023","volume":"127","author":"JK McGill","year":"2006","unstructured":"McGill JK, Beal MF (2006) PGC-1alpha, a new therapeutic target in Huntington\u2019s disease? Cell 127:465\u2013468","journal-title":"Cell"},{"key":"9766_CR77","doi-asserted-by":"crossref","unstructured":"Metzler M, Chen N, Helgason CD, Graham RK, Nichol K, McCutcheon K, Nasir J, Humphries RK, Raymond LA, Hayden MR (1999) Life without huntingtin: normal differentiation into functional neurons. J Neurochem. 72(3):1009\u201318","DOI":"10.1046\/j.1471-4159.1999.0721009.x"},{"key":"9766_CR78","doi-asserted-by":"crossref","first-page":"1830","DOI":"10.1523\/JNEUROSCI.21-06-01830.2001","volume":"21","author":"LM Mende-Mueller","year":"2001","unstructured":"Mende-Mueller LM, Toneff T, Hwang SR, Chesselet MF, Hook VY (2001) Tissue-specific proteolysis of Huntingtin (htt) in human brain: evidence of enhanced levels of N- and C-terminal htt fragments in Huntington\u2019s disease striatum. J Neurosci 21:1830\u20131837","journal-title":"J Neurosci"},{"key":"9766_CR79","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1016\/S0301-0082(00)00014-9","volume":"63","author":"MG Murer","year":"2001","unstructured":"Murer MG, Yan Q, Raisman-Vozari R (2001) Brain-derived neurotrophic factor in the control human brain, and in Alzheimer\u2019s disease and Parkinson\u2019s disease. Prog Neurobiol 63:71\u2013124","journal-title":"Prog Neurobiol"},{"key":"9766_CR80","doi-asserted-by":"crossref","unstructured":"Nakao N, Kokaia Z, Odin P, Lindvall O (1995) Protective effects of BDNF and NT-3 but not PDGF against hypoglycemic injury to cultured striatal neurons. Exp Neurol. 131(1):1\u201310","DOI":"10.1016\/0014-4886(95)90002-0"},{"key":"9766_CR81","doi-asserted-by":"crossref","first-page":"25060","DOI":"10.1074\/jbc.M501599200","volume":"280","author":"M Neuspiel","year":"2005","unstructured":"Neuspiel M, Zunino R, Gangaraju S, Rippstein P, McBride H (2005) Activated mitofusin 2 signals mitochondrial fusion, interferes with Bax activation, and reduces susceptibility to radical induced depolarization. J Biol Chem 280:25060\u201325070","journal-title":"J Biol Chem"},{"key":"9766_CR82","doi-asserted-by":"crossref","first-page":"468","DOI":"10.1016\/S0006-8993(98)01312-2","volume":"818","author":"JR O\u2019Kusky","year":"1999","unstructured":"O\u2019Kusky JR, Nasir J, Cicchetti F, Parent A, Hayden MR (1999) Neuronal degeneration in the basal ganglia and loss of pallido-subthalamic synapses in mice with targeted disruption of the Huntington\u2019s disease gene. Brain Res 818:468\u2013479","journal-title":"Brain Res"},{"key":"9766_CR83","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"},{"key":"9766_CR84","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 5:731\u2013736","journal-title":"Nat Neurosci"},{"key":"9766_CR85","doi-asserted-by":"crossref","first-page":"652","DOI":"10.1111\/jnc.13165","volume":"134","author":"JJ Pellman","year":"2015","unstructured":"Pellman JJ, Hamilton J, Brustovetsky T, Brustovetsky N (2015) Ca(2+) handling in isolated brain mitochondria and cultured neurons derived from the YAC128 mouse model of Huntington\u2019s disease. J Neurochem 134:652\u2013667","journal-title":"J Neurochem"},{"key":"9766_CR86","doi-asserted-by":"crossref","first-page":"5355","DOI":"10.1073\/pnas.91.12.5355","volume":"91","author":"MF Perutz","year":"1994","unstructured":"Perutz MF, Johnson T, Suzuki M, Finch JT (1994) Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases. Proc Natl Acad Sci U S A 91:5355\u20135358","journal-title":"Proc Natl Acad Sci U S A"},{"key":"9766_CR87","doi-asserted-by":"crossref","first-page":"771","DOI":"10.1038\/nature02583","volume":"429","author":"F Picard","year":"2004","unstructured":"Picard F, Kurtev M, Chung N, Topark-Ngarm A, Senawong T, Machado De Oliveira R, Leid M, McBurney MW, Guarente L (2004) Sirt1 promotes fat mobilization in white adipocytes by repressing PPAR-gamma. Nature 429:771\u2013776","journal-title":"Nature"},{"key":"9766_CR88","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1016\/j.neuron.2014.12.007","volume":"85","author":"AM Pickrell","year":"2015","unstructured":"Pickrell AM, Youle RJ (2015) The roles of PINK1, parkin, and mitochondrial fidelity in Parkinson\u2019s disease. Neuron 85:257\u2013273","journal-title":"Neuron"},{"key":"9766_CR89","doi-asserted-by":"crossref","first-page":"2809","DOI":"10.1523\/JNEUROSCI.20-08-02809.2000","volume":"20","author":"T Pizzorusso","year":"2000","unstructured":"Pizzorusso T, Ratto GM, Putignano E, Maffei L (2000) Brain-derived neurotrophic factor causes cAMP response element-binding protein phosphorylation in absence of calcium increases in slices and cultured neurons from rat visual cortex. J Neurosci 20:2809\u20132816","journal-title":"J Neurosci"},{"key":"9766_CR90","doi-asserted-by":"crossref","first-page":"1107","DOI":"10.1093\/hmg\/11.9.1107","volume":"11","author":"B Ravikumar","year":"2002","unstructured":"Ravikumar B, Duden R, Rubinsztein DC (2002) Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy. Hum Mol Genet 11:1107\u20131117","journal-title":"Hum Mol Genet"},{"key":"9766_CR91","doi-asserted-by":"crossref","first-page":"393","DOI":"10.2174\/156720511795745401","volume":"8","author":"PH Reddy","year":"2011","unstructured":"Reddy PH, Reddy TP (2011) Mitochondria as a therapeutic target for aging and neurodegenerative diseases. Curr Alzheimer Res 8:393\u2013409","journal-title":"Curr Alzheimer Res"},{"key":"9766_CR92","doi-asserted-by":"crossref","unstructured":"Rigamonti D, Bauer JH, De-Fraja C, Conti L, Sipione S, Sciorati C, Clementi E, Hackam A, Hayden MR, Li Y, Cooper JK, Ross CA, Govoni S, Vincenz C, Cattaneo E (2000) Wild-type huntingtin protects from apoptosis upstream of caspase-3. J Neurosci. 15;20(10):3705\u201313","DOI":"10.1523\/JNEUROSCI.20-10-03705.2000"},{"key":"9766_CR93","first-page":"545","volume":"5","author":"M Rinnerthaler","year":"2015","unstructured":"Rinnerthaler M, Bischof J, Streubel MK, Trost A, Richter K (2015) Oxidative stress in aging human skin. Biomol Ther 5:545\u2013589","journal-title":"Biomol Ther"},{"key":"9766_CR94","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/S1474-4422(10)70245-3","volume":"10","author":"CA Ross","year":"2011","unstructured":"Ross CA, Tabrizi SJ (2011) Huntington\u2019s disease: from molecular pathogenesis to clinical treatment. Lancet Neurol 10:83\u201398","journal-title":"Lancet Neurol"},{"key":"9766_CR95","doi-asserted-by":"crossref","first-page":"e7","DOI":"10.1038\/cddis.2009.6","volume":"1","author":"J Sassone","year":"2010","unstructured":"Sassone J, Colciago C, Marchi P, Ascardi C, Alberti L, Di Pardo A, Zippel R, Sipione S, Silani V, Ciammola A (2010) Mutant Huntingtin induces activation of the Bcl-2\/adenovirus E1B 19-kDa interacting protein (BNip3). Cell Death Dis 1:e7","journal-title":"Cell Death Dis"},{"key":"9766_CR96","doi-asserted-by":"crossref","first-page":"5489","DOI":"10.1038\/sj.onc.1210616","volume":"26","author":"LR Saunders","year":"2007","unstructured":"Saunders LR, Verdin E (2007) Sirtuins: critical regulators at the crossroads between cancer and aging. Oncogene 26:5489\u20135504","journal-title":"Oncogene"},{"key":"9766_CR97","doi-asserted-by":"crossref","first-page":"6472","DOI":"10.1073\/pnas.0308686101","volume":"101","author":"SN Schreiber","year":"2004","unstructured":"Schreiber SN, Emter R, Hock MB, Knutti D, Cardenas J, Podvinec M, Oakeley EJ, Kralli A (2004) The estrogen-related receptor alpha (ERRalpha) functions in PPARgamma coactivator 1alpha (PGC-1alpha)-induced mitochondrial biogenesis. Proc Natl Acad Sci U S A 101:6472\u20136477","journal-title":"Proc Natl Acad Sci U S A"},{"key":"9766_CR98","doi-asserted-by":"crossref","unstructured":"Sheng M, Kim MJ (2002) Postsynaptic signaling and plasticity mechanisms. Science. 25;298(5594):776\u201380","DOI":"10.1126\/science.1075333"},{"key":"9766_CR99","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1093\/cvr\/cvv097","volume":"106","author":"LK Seidlmayer","year":"2015","unstructured":"Seidlmayer LK, Juettner VV, Kettlewell S, Pavlov EV, Blatter LA, Dedkova EN (2015) Distinct mPTP activation mechanisms in ischaemia-reperfusion: contributions of Ca2+, ROS, pH, and inorganic polyphosphate. Cardiovasc Res 106:237\u2013248","journal-title":"Cardiovasc Res"},{"key":"9766_CR100","doi-asserted-by":"crossref","first-page":"570","DOI":"10.1016\/j.molcel.2008.08.002","volume":"31","author":"C Sheridan","year":"2008","unstructured":"Sheridan C, Delivani P, Cullen SP, Martin SJ (2008) Bax- or Bak-induced mitochondrial fission can be uncoupled from cytochrome C release. Mol Cell 31:570\u2013585","journal-title":"Mol Cell"},{"key":"9766_CR101","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.neuron.2012.05.024","volume":"75","author":"DI Shirasaki","year":"2012","unstructured":"Shirasaki DI, Greiner ER, Al-Ramahi I, Gray M, Boontheung P, Geschwind DH, Botas J, Coppola G, Horvath S, Loo JA, Yang XW (2012) Network organization of the huntingtin proteomic interactome in mammalian brain. Neuron 75:41\u201357","journal-title":"Neuron"},{"key":"9766_CR102","doi-asserted-by":"crossref","first-page":"1438","DOI":"10.1093\/hmg\/ddr024","volume":"20","author":"U Shirendeb","year":"2011","unstructured":"Shirendeb U, Reddy AP, Manczak M, Calkins MJ, Mao P, Tagle DA, Reddy PH (2011) Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington\u2019s disease: implications for selective neuronal damage. Hum Mol Genet 20:1438\u20131455","journal-title":"Hum Mol Genet"},{"key":"9766_CR103","doi-asserted-by":"crossref","first-page":"1953","DOI":"10.1093\/hmg\/11.17.1953","volume":"11","author":"S Sipione","year":"2002","unstructured":"Sipione S, Rigamonti D, Valenza M, Zuccato C, Conti L, Pritchard J, Kooperberg C, Olson JM, Cattaneo E (2002) Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses. Hum Mol Genet 11:1953\u20131965","journal-title":"Hum Mol Genet"},{"key":"9766_CR104","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1083\/jcb.144.2.281","volume":"144","author":"EA Slee","year":"1999","unstructured":"Slee EA, Harte MT, Kluck RM, Wolf BB, Casiano CA, Newmeyer DD, Wang HG, Reed JC, Nicholson DW, Alnemri ES, Green DR, Martin SJ (1999) Ordering the cytochrome c-initiated caspase cascade: hierarchical activation of caspases-2, -3, -6, -7, -8, and -10 in a caspase-9-dependent manner. J Cell Biol 144:281\u2013292","journal-title":"J Cell Biol"},{"key":"9766_CR105","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1023\/A:1022534709672","volume":"24","author":"L Sokoloff","year":"1999","unstructured":"Sokoloff L (1999) Energetics of functional activation in neural tissues. Neurochem Res 24:321\u2013329","journal-title":"Neurochem Res"},{"key":"9766_CR106","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1038\/nm.2313","volume":"17","author":"W Song","year":"2011","unstructured":"Song W, Chen J, Petrilli A, Liot G, Klinglmayr E, Zhou Y, Poquiz P, Tjong J, Pouladi MA, Hayden MR, Masliah E, Ellisman M, Rouiller I, Schwarzenbacher R, Bossy B, Perkins G, Bossy-Wetzel E (2011) Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity. Nat Med 17:377\u2013382","journal-title":"Nat Med"},{"key":"9766_CR107","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1038\/nrn1007","volume":"4","author":"C Soto","year":"2003","unstructured":"Soto C (2003) Unfolding the role of protein misfolding in neurodegenerative diseases. Nat Rev Neurosci 4:49\u201360","journal-title":"Nat Rev Neurosci"},{"key":"9766_CR108","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1126\/science.1092194","volume":"304","author":"JS Steffan","year":"2004","unstructured":"Steffan JS, Agrawal N, Pallos J, Rockabrand E, Trotman LC, Slepko N, Illes K, Lukacsovich T, Zhu YZ, Cattaneo E, Pandolfi PP, Thompson LM, Marsh JL (2004) SUMO modification of Huntingtin and Huntington\u2019s disease pathology. Science 304:100\u2013104","journal-title":"Science"},{"key":"9766_CR109","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 97:6763\u20136768","journal-title":"Proc Natl Acad Sci U S A"},{"key":"9766_CR110","doi-asserted-by":"crossref","first-page":"1279","DOI":"10.1038\/nsmb.1700","volume":"16","author":"S Tam","year":"2009","unstructured":"Tam S, Spiess C, Auyeung W, Joachimiak L, Chen B, Poirier MA, Frydman J (2009) The chaperonin TRiC blocks a huntingtin sequence element that promotes the conformational switch to aggregation. Nat Struct Mol Biol 16:1279\u20131285","journal-title":"Nat Struct Mol Biol"},{"key":"9766_CR111","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1016\/S0006-291X(02)00881-1","volume":"296","author":"S Terada","year":"2002","unstructured":"Terada S, Goto M, Kato M, Kawanaka K, Shimokawa T, Tabata I (2002) Effects of low-intensity prolonged exercise on PGC-1 mRNA expression in rat epitrochlearis muscle. Biochem Biophys Res Commun 296:350\u2013354","journal-title":"Biochem Biophys Res Commun"},{"key":"9766_CR112","doi-asserted-by":"crossref","first-page":"8195","DOI":"10.1128\/MCB.24.18.8195-8209.2004","volume":"24","author":"E Trushina","year":"2004","unstructured":"Trushina E, Dyer RB, Badger JD, Ure D, Eide L, Tran DD, Vrieze BT, Legendre-Guillemin V, McPherson PS, Mandavilli BS, Van Houten B, Zeitlin S, McNiven M, Aebersold R, Hayden M, Parisi JE, Seeberg E, Dragatsis I, Doyle K, Bender A et al (2004) Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro. Mol Cell Biol 24:8195\u20138209","journal-title":"Mol Cell Biol"},{"key":"9766_CR113","doi-asserted-by":"crossref","first-page":"1868","DOI":"10.1128\/MCB.20.5.1868-1876.2000","volume":"20","author":"RB Vega","year":"2000","unstructured":"Vega RB, Huss JM, Kelly DP (2000) The coactivator PGC-1 cooperates with peroxisome proliferator-activated receptor alpha in transcriptional control of nuclear genes encoding mitochondrial fatty acid oxidation enzymes. Mol Cell Biol 20:1868\u20131876","journal-title":"Mol Cell Biol"},{"key":"9766_CR114","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1111\/febs.13175","volume":"282","author":"JA Villena","year":"2015","unstructured":"Villena JA (2015) New insights into PGC-1 coactivators: redefining their role in the regulation of mitochondrial function and beyond. FEBS J 282:647\u2013672","journal-title":"FEBS J"},{"key":"9766_CR115","doi-asserted-by":"crossref","first-page":"12317","DOI":"10.1073\/pnas.1007088107","volume":"107","author":"N Volakakis","year":"2010","unstructured":"Volakakis N, Kadkhodaei B, Joodmardi E, Wallis K, Panman L, Silvaggi J, Spiegelman BM, Perlmann T (2010) NR4A orphan nuclear receptors as mediators of CREB-dependent neuroprotection. Proc Natl Acad Sci U S A 107:12317\u201312322","journal-title":"Proc Natl Acad Sci U S A"},{"key":"9766_CR116","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1097\/00005072-199805000-00001","volume":"57","author":"JP Vonsattel","year":"1998","unstructured":"Vonsattel JP, DiFiglia M (1998) Huntington disease. J Neuropathol Exp Neurol 57:369\u2013384","journal-title":"J Neuropathol Exp Neurol"},{"key":"9766_CR117","doi-asserted-by":"crossref","unstructured":"Walker FO (2007) Huntington\u2019s disease. Lancet. 369(9557):218\u201328","DOI":"10.1016\/S0140-6736(07)60111-1"},{"key":"9766_CR118","doi-asserted-by":"crossref","first-page":"893","DOI":"10.1016\/j.cell.2011.10.018","volume":"147","author":"X Wang","year":"2011","unstructured":"Wang X, Winter D, Ashrafi G, Schlehe J, Wong YL, Selkoe D, Rice S, Steen J, LaVoie MJ, Schwarz TL (2011) PINK1 and Parkin target Miro for phosphorylation and degradation to arrest mitochondrial motility. Cell 147:893\u2013906","journal-title":"Cell"},{"key":"9766_CR119","doi-asserted-by":"crossref","first-page":"1569","DOI":"10.1093\/hmg\/ddi165","volume":"14","author":"SC Warby","year":"2005","unstructured":"Warby SC, Chan EY, Metzler M, Gan L, Singaraja RR, Crocker SF, Robertson HA, Hayden MR (2005) Huntingtin phosphorylation on serine 421 is significantly reduced in the striatum and by polyglutamine expansion in vivo. Hum Mol Genet 14:1569\u20131577","journal-title":"Hum Mol Genet"},{"issue":"5","key":"9766_CR120","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/j.cmet.2006.10.004","volume":"4","author":"P Weydt","year":"2006","unstructured":"Weydt P, Pineda VV, Torrence AE, Libby RT, Satterfield TF, Lazarowski ER, Gilbert ML, Morton GJ, Bammler TK, Strand AD, Cui L, Beyer RP, Easley CN, Smith AC, Krainc D, Luquet S, Sweet IR, Schwartz MW, La Spada AR (2006) Thermoregulatory and metabolic defects in Huntington\u2019s disease transgenic mice implicate PGC-1alpha in Huntington\u2019s disease neurodegeneration. Cell Metab 4(5):349\u2013362","journal-title":"Cell Metab"},{"key":"9766_CR121","doi-asserted-by":"crossref","unstructured":"White JK, Auerbach W, Duyao MP, Vonsattel JP, Gusella JF, Joyner AL, MacDonald ME (1997) Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion. Nat Genet. 17(4):404\u201310","DOI":"10.1038\/ng1297-404"},{"key":"9766_CR122","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1016\/j.tins.2008.07.004","volume":"31","author":"AJ Williams","year":"2008","unstructured":"Williams AJ, Paulson HL (2008) Polyglutamine neurodegeneration: protein misfolding revisited. Trends Neurosci 31:521\u2013528","journal-title":"Trends Neurosci"},{"key":"9766_CR123","doi-asserted-by":"crossref","first-page":"969","DOI":"10.1016\/0378-4274(95)03532-X","volume":"82-83","author":"CC Winterbourn","year":"1995","unstructured":"Winterbourn CC (1995) Toxicity of iron and hydrogen peroxide: the Fenton reaction. Toxicol Lett 82-83:969\u2013974","journal-title":"Toxicol Lett"},{"key":"9766_CR124","doi-asserted-by":"crossref","first-page":"1293","DOI":"10.1523\/JNEUROSCI.1870-13.2014","volume":"34","author":"YC Wong","year":"2014","unstructured":"Wong YC, Holzbaur EL (2014) The regulation of autophagosome dynamics by huntingtin and HAP1 is disrupted by expression of mutant huntingtin, leading to defective cargo degradation. J Neurosci 34:1293\u20131305","journal-title":"J Neurosci"},{"key":"9766_CR125","doi-asserted-by":"crossref","first-page":"14379","DOI":"10.1073\/pnas.0606714103","volume":"103","author":"Z Wu","year":"2006","unstructured":"Wu Z, Huang X, Feng Y, Handschin C, Gullicksen PS, Bare O, Labow M, Spiegelman B, Stevenson SC (2006) Transducer of regulated CREB-binding proteins (TORCs) induce PGC-1alpha transcription and mitochondrial biogenesis in muscle cells. Proc Natl Acad Sci U S A 103:14379\u201314384","journal-title":"Proc Natl Acad Sci U S A"},{"key":"9766_CR126","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/S0092-8674(00)80611-X","volume":"98","author":"Z Wu","year":"1999","unstructured":"Wu Z, Puigserver P, Andersson U, Zhang C, Adelmant G, Mootha V, Troy A, Cinti S, Lowell B, Scarpulla RC, Spiegelman BM (1999) Mechanisms controlling mitochondrial biogenesis and respiration through the thermogenic coactivator PGC-1. Cell 98:115\u2013124","journal-title":"Cell"},{"key":"9766_CR127","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1038\/nrm2308","volume":"9","author":"RJ Youle","year":"2008","unstructured":"Youle RJ, Strasser A (2008) The BCL-2 protein family: opposing activities that mediate cell death. Nat Rev Mol Cell Biol 9:47\u201359","journal-title":"Nat Rev Mol Cell Biol"},{"key":"9766_CR128","doi-asserted-by":"crossref","first-page":"1241","DOI":"10.1016\/j.cell.2005.10.030","volume":"123","author":"W Zhai","year":"2005","unstructured":"Zhai W, Jeong H, Cui L, Krainc D, Tjian R (2005) In vitro analysis of huntingtin-mediated transcriptional repression reveals multiple transcription factor targets. Cell 123:1241\u20131253","journal-title":"Cell"},{"key":"9766_CR129","doi-asserted-by":"crossref","first-page":"53963","DOI":"10.1074\/jbc.M406028200","volume":"279","author":"Y Zhang","year":"2004","unstructured":"Zhang Y, Ma K, Song S, Elam MB, Cook GA, Park EA (2004) Peroxisomal proliferator-activated receptor-gamma coactivator-1 alpha (PGC-1 alpha) enhances the thyroid hormone induction of carnitine palmitoyltransferase I (CPT-I alpha). J Biol Chem 279:53963\u201353971","journal-title":"J Biol Chem"},{"key":"9766_CR130","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1016\/j.pneurobio.2007.01.003","volume":"81","author":"C Zuccato","year":"2007","unstructured":"Zuccato C, Cattaneo E (2007) Role of brain-derived neurotrophic factor in Huntington\u2019s disease. Prog Neurobiol 81:294\u2013330","journal-title":"Prog Neurobiol"},{"key":"9766_CR131","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1126\/science.1059581","volume":"293","author":"C Zuccato","year":"2001","unstructured":"Zuccato C, Ciammola A, Rigamonti D, Leavitt BR, Goffredo D, Conti L, MacDonald ME, Friedlander RM, Silani V, Hayden MR, Timmusk T, Sipione S, Cattaneo E (2001) Loss of huntingtin-mediated BDNF gene transcription in Huntington\u2019s disease. Science 293:493\u2013498","journal-title":"Science"},{"key":"9766_CR132","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1038\/ng1341","volume":"36","author":"S Z\u00fcchner","year":"2004","unstructured":"Z\u00fcchner S, Mersiyanova IV, Muglia M, Bissar-Tadmouri N, Rochelle J, Dadali EL, Zappia M, Nelis E, Patitucci A, Senderek J, Parman Y, Evgrafov O, Jonghe PD, Takahashi Y, Tsuji S, Pericak-Vance MA, Quattrone A, Battaloglu E, Polyakov AV, Timmerman V et al (2004) Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A. Nat Genet 36:449\u2013451","journal-title":"Nat Genet"}],"container-title":["Neurotoxicity Research"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s12640-017-9766-1\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s12640-017-9766-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s12640-017-9766-1.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,6,19]],"date-time":"2025-06-19T21:29:51Z","timestamp":1750368591000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s12640-017-9766-1"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,6,21]]},"references-count":132,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2017,10]]}},"alternative-id":["9766"],"URL":"https:\/\/doi.org\/10.1007\/s12640-017-9766-1","relation":{},"ISSN":["1029-8428","1476-3524"],"issn-type":[{"value":"1029-8428","type":"print"},{"value":"1476-3524","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,6,21]]}}}