{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T17:00:14Z","timestamp":1790182814979,"version":"4.1.0"},"reference-count":58,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2015,12,7]],"date-time":"2015-12-07T00:00:00Z","timestamp":1449446400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Nat Med"],"published-print":{"date-parts":[[2016,1]]},"DOI":"10.1038\/nm.4003","type":"journal-article","created":{"date-parts":[[2015,12,7]],"date-time":"2015-12-07T11:22:36Z","timestamp":1449487356000},"page":"37-45","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":104,"title":["PPAR-\u03b4 is repressed in Huntington's disease, is required for normal neuronal function and can be targeted therapeutically"],"prefix":"10.1038","volume":"22","author":[{"given":"Audrey S","family":"Dickey","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor V","family":"Pineda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taiji","family":"Tsunemi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick P","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen C","family":"Miranda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen K","family":"Gilmore-Hall","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Lomas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kunal R","family":"Sampat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"Buttgereit","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark-Joseph Manalang","family":"Torres","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"April L","family":"Flores","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7630-1403","authenticated-orcid":false,"given":"Martin","family":"Arreola","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1038-192X","authenticated-orcid":false,"given":"Nicolas","family":"Arbez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sergey S","family":"Akimov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terry","family":"Gaasterland","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo R","family":"Lazarowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher A","family":"Ross","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gene W","family":"Yeo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bryce L","family":"Sopher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gavin K","family":"Magnuson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony B","family":"Pinkerton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eliezer","family":"Masliah","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Albert R","family":"La Spada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2015,12,7]]},"reference":[{"key":"BFnm4003_CR1","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1146\/annurev.med.53.082901.104018","volume":"53","author":"J Berger","year":"2002","unstructured":"Berger, J. & Moller, D.E. The mechanisms of action of PPARs. Annu. Rev. Med. 53, 409\u2013435 (2002).","journal-title":"Annu. Rev. Med."},{"key":"BFnm4003_CR2","doi-asserted-by":"crossref","first-page":"1319","DOI":"10.2337\/diab.46.8.1319","volume":"46","author":"D Auboeuf","year":"1997","unstructured":"Auboeuf, D. et al. Tissue distribution and quantification of the expression of mRNAs of peroxisome proliferator-activated receptors and liver X receptor-\u03b1 in humans: no alteration in adipose tissue of obese and NIDDM patients. Diabetes 46, 1319\u20131327 (1997).","journal-title":"Diabetes"},{"key":"BFnm4003_CR3","doi-asserted-by":"crossref","first-page":"7355","DOI":"10.1073\/pnas.91.15.7355","volume":"91","author":"SA Kliewer","year":"1994","unstructured":"Kliewer, S.A. et al. Differential expression and activation of a family of murine peroxisome proliferator-activated receptors. Proc. Natl. Acad. Sci. USA 91, 7355\u20137359 (1994).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnm4003_CR4","doi-asserted-by":"crossref","first-page":"2299","DOI":"10.1096\/fj.03-0269fje","volume":"17","author":"S Luquet","year":"2003","unstructured":"Luquet, S. et al. Peroxisome proliferator-activated receptor\u2013\u03b4 controls muscle development and oxidative capability. FASEB J. 17, 2299\u20132301 (2003).","journal-title":"FASEB J."},{"key":"BFnm4003_CR5","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/j.cmet.2006.10.003","volume":"4","author":"M Schuler","year":"2006","unstructured":"Schuler, M. et al. PGC1-\u03b1 expression is controlled in skeletal muscles by PPAR-\u03b2, whose ablation results in fiber-type switching, obesity and type 2 diabetes. Cell Metab. 4, 407\u2013414 (2006).","journal-title":"Cell Metab."},{"key":"BFnm4003_CR6","doi-asserted-by":"crossref","first-page":"e294","DOI":"10.1371\/journal.pbio.0020294","volume":"2","author":"YX Wang","year":"2004","unstructured":"Wang, Y.X. et al. Regulation of muscle fiber type and running endurance by PPAR-\u03b4. PLoS Biol. 2, e294 (2004).","journal-title":"PLoS Biol."},{"key":"BFnm4003_CR7","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1016\/j.cell.2008.06.051","volume":"134","author":"VA Narkar","year":"2008","unstructured":"Narkar, V.A. et al. AMPK and PPAR-\u03b4 agonists are exercise mimetics. Cell 134, 405\u2013415 (2008).","journal-title":"Cell"},{"key":"BFnm4003_CR8","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1016\/j.bbrc.2008.04.086","volume":"371","author":"EE Girroir","year":"2008","unstructured":"Girroir, E.E. et al. Quantitative expression patterns of peroxisome proliferator-activated receptor-\u03b2\/\u03b4 (PPAR-\u03b2\/\u03b4) protein in mice. Biochem. Biophys. Res. Commun. 371, 456\u2013461 (2008).","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"BFnm4003_CR9","doi-asserted-by":"crossref","first-page":"1048","DOI":"10.1212\/WNL.49.4.1048","volume":"49","author":"MA Nance","year":"1997","unstructured":"Nance, M.A. Genetic testing of children at risk for Huntington\u2019s disease. Neurology 49, 1048\u20131053 (1997).","journal-title":"Neurology"},{"key":"BFnm4003_CR10","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1111\/j.1750-3639.1997.tb00898.x","volume":"7","author":"CA Ross","year":"1997","unstructured":"Ross, C.A. et al. Huntington\u2019s disease and dentatorubral-pallidoluysian atrophy: proteins, pathogenesis and pathology. Brain Pathol. 7, 1003\u20131016 (1997).","journal-title":"Brain Pathol."},{"issue":"6","key":"BFnm4003_CR11","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","volume":"72","author":"M MACDONALD","year":"1993","unstructured":"The Huntington\u2019s Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington\u2019s disease chromosomes. Cell 72, 971\u2013983 (1993).","journal-title":"Cell"},{"key":"BFnm4003_CR12","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1038\/nrg2748","volume":"11","author":"AR La Spada","year":"2010","unstructured":"La Spada, A.R. & Taylor, J.P. Repeat expansion disease: progress and puzzles in disease pathogenesis. Nat. Rev. Genet. 11, 247\u2013258 (2010).","journal-title":"Nat. Rev. Genet."},{"key":"BFnm4003_CR13","doi-asserted-by":"crossref","first-page":"4181","DOI":"10.1523\/JNEUROSCI.13-10-04181.1993","volume":"13","author":"MF Beal","year":"1993","unstructured":"Beal, M.F. et al. Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid. J. Neurosci. 13, 4181\u20134192 (1993).","journal-title":"J. Neurosci."},{"key":"BFnm4003_CR14","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1038\/nature05292","volume":"443","author":"MT Lin","year":"2006","unstructured":"Lin, M.T. & Beal, M.F. Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases. Nature 443, 787\u2013795 (2006).","journal-title":"Nature"},{"key":"BFnm4003_CR15","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, M.E. Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. Cell 95, 55\u201366 (1998).","journal-title":"Cell"},{"key":"BFnm4003_CR16","doi-asserted-by":"crossref","first-page":"2183","DOI":"10.1101\/gad.1436506","volume":"20","author":"BE Riley","year":"2006","unstructured":"Riley, B.E. & Orr, H.T. Polyglutamine neurodegenerative diseases and regulation of transcription: assembling the puzzle. Genes Dev. 20, 2183\u20132192 (2006).","journal-title":"Genes Dev."},{"key":"BFnm4003_CR17","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. et al. Transcriptional repression of PGC-1\u03b1 by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell 127, 59\u201369 (2006).","journal-title":"Cell"},{"key":"BFnm4003_CR18","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. et al. Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1\u03b1\u2013null mice. Cell 119, 121\u2013135 (2004).","journal-title":"Cell"},{"key":"BFnm4003_CR19","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. et al. Thermoregulatory and metabolic defects in Huntington\u2019s disease transgenic mice implicate PGC-1\u03b1 in Huntington\u2019s disease neurodegeneration. Cell Metab. 4, 349\u2013362 (2006).","journal-title":"Cell Metab."},{"key":"BFnm4003_CR20","doi-asserted-by":"crossref","first-page":"142ra97","DOI":"10.1126\/scitranslmed.3003799","volume":"4","author":"T Tsunemi","year":"2012","unstructured":"Tsunemi, T. et al. PGC-1\u03b1 rescues Huntington\u2019s disease proteotoxicity by preventing oxidative stress and promoting TFEB function. Sci. Transl. Med. 4, 142ra97 (2012).","journal-title":"Sci. Transl. Med."},{"key":"BFnm4003_CR21","doi-asserted-by":"crossref","first-page":"6182","DOI":"10.1523\/JNEUROSCI.0857-08.2008","volume":"28","author":"M Gray","year":"2008","unstructured":"Gray, M. et al. Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice. J. Neurosci. 28, 6182\u20136195 (2008).","journal-title":"J. Neurosci."},{"key":"BFnm4003_CR22","doi-asserted-by":"crossref","first-page":"4043","DOI":"10.1093\/hmg\/ddq322","volume":"19","author":"MC Chiang","year":"2010","unstructured":"Chiang, M.C. et al. Modulation of energy deficiency in Huntington\u2019s disease via activation of the peroxisome proliferator-activated receptor\u2013\u03b3. Hum. Mol. Genet. 19, 4043\u20134058 (2010).","journal-title":"Hum. Mol. Genet."},{"key":"BFnm4003_CR23","doi-asserted-by":"crossref","first-page":"e30406","DOI":"10.1371\/journal.pone.0030406","volume":"7","author":"YN Jin","year":"2012","unstructured":"Jin, Y.N., Hwang, W.Y., Jo, C. & Johnson, G.V. Metabolic state determines sensitivity to cellular stress in Huntington disease: normalization by activation of PPAR-\u03b3. PLoS One 7, e30406 (2012).","journal-title":"PLoS One"},{"key":"BFnm4003_CR24","doi-asserted-by":"crossref","first-page":"25628","DOI":"10.1074\/jbc.M804291200","volume":"283","author":"RA Quintanilla","year":"2008","unstructured":"Quintanilla, R.A., Jin, Y.N., Fuenzalida, K., Bronfman, M. & Johnson, G.V. Rosiglitazone treatment prevents mitochondrial dysfunction in mutant huntingtin\u2013expressing cells: possible role of peroxisome proliferator-activated receptor\u2013\u03b3 (PPAR-\u03b3) in the pathogenesis of Huntington disease. J. Biol. Chem. 283, 25628\u201325637 (2008).","journal-title":"J. Biol. Chem."},{"key":"BFnm4003_CR25","doi-asserted-by":"crossref","first-page":"38768","DOI":"10.1074\/jbc.M006450200","volume":"275","author":"C Bastie","year":"2000","unstructured":"Bastie, C., Luquet, S., Holst, D., Jehl-Pietri, C. & Grimaldi, P.A. Alterations of peroxisome proliferator-activated receptor\u2013\u03b4 activity affect fatty acid\u2013controlled adipose differentiation. J. Biol. Chem. 275, 38768\u201338773 (2000).","journal-title":"J. Biol. Chem."},{"key":"BFnm4003_CR26","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1016\/S0014-4827(03)00179-4","volume":"288","author":"D Holst","year":"2003","unstructured":"Holst, D., Luquet, S., Kristiansen, K. & Grimaldi, P.A. Roles of peroxisome proliferator-activated receptors\u2013\u03b4 and \u2013\u03b3 in myoblast transdifferentiation. Exp. Cell Res. 288, 168\u2013176 (2003).","journal-title":"Exp. Cell Res."},{"key":"BFnm4003_CR27","doi-asserted-by":"crossref","first-page":"5119","DOI":"10.1128\/MCB.20.14.5119-5128.2000","volume":"20","author":"JM Peters","year":"2000","unstructured":"Peters, J.M. et al. Growth, adipose, brain and skin alterations resulting from targeted disruption of the mouse peroxisome proliferator-activated receptor\u2013\u03b2(\u03b4). Mol. Cell. Biol. 20, 5119\u20135128 (2000).","journal-title":"Mol. Cell. Biol."},{"key":"BFnm4003_CR28","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1038\/12703","volume":"23","author":"F Tronche","year":"1999","unstructured":"Tronche, F. et al. Disruption of the glucocorticoid receptor gene in the nervous system results in reduced anxiety. Nat. Genet. 23, 99\u2013103 (1999).","journal-title":"Nat. Genet."},{"key":"BFnm4003_CR29","first-page":"e1787","volume":"39","author":"SJ Guyenet","year":"2010","unstructured":"Guyenet, S.J. et al. A simple composite phenotype scoring system for evaluating mouse models of cerebellar ataxia. J. Vis. Exp. 39, e1787 (2010).","journal-title":"J. Vis. Exp."},{"key":"BFnm4003_CR30","doi-asserted-by":"crossref","first-page":"406","DOI":"10.1093\/hmg\/ddr475","volume":"21","author":"UP Shirendeb","year":"2012","unstructured":"Shirendeb, U.P. et al. Mutant huntingtin\u2019s interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington\u2019s disease. Hum. Mol. Genet. 21, 406\u2013420 (2012).","journal-title":"Hum. Mol. Genet."},{"key":"BFnm4003_CR31","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1038\/nm.2313","volume":"17","author":"W Song","year":"2011","unstructured":"Song, W. et al. Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein 1 and increases its enzymatic activity. Nat. Med. 17, 377\u2013382 (2011).","journal-title":"Nat. Med."},{"key":"BFnm4003_CR32","doi-asserted-by":"crossref","first-page":"15254","DOI":"10.1073\/pnas.0601758103","volume":"103","author":"MT Dang","year":"2006","unstructured":"Dang, M.T. et al. Disrupted motor learning and long-term synaptic plasticity in mice lacking NMDAR1 in the striatum. Proc. Natl. Acad. Sci. USA 103, 15254\u201315259 (2006).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnm4003_CR33","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1016\/0006-8993(87)90694-9","volume":"411","author":"RJ Ferrante","year":"1987","unstructured":"Ferrante, R.J., Beal, M.F., Kowall, N.W., Richardson, E.P. Jr. & Martin, J.B. Sparing of acetylcholinesterase-containing striatal neurons in Huntington\u2019s disease. Brain Res. 411, 162\u2013166 (1987).","journal-title":"Brain Res."},{"key":"BFnm4003_CR34","doi-asserted-by":"crossref","first-page":"1691","DOI":"10.1002\/mds.25624","volume":"28","author":"A Reiner","year":"2013","unstructured":"Reiner, A. et al. Striatal parvalbuminergic neurons are lost in Huntington\u2019s disease: implications for dystonia. Mov. Disord. 28, 1691\u20131699 (2013).","journal-title":"Mov. Disord."},{"key":"BFnm4003_CR35","doi-asserted-by":"crossref","first-page":"965","DOI":"10.1093\/hmg\/ddl013","volume":"15","author":"A Hodges","year":"2006","unstructured":"Hodges, A. et al. Regional and cellular gene expression changes in human Huntington\u2019s disease brain. Hum. Mol. Genet. 15, 965\u2013977 (2006).","journal-title":"Hum. Mol. Genet."},{"key":"BFnm4003_CR36","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1021\/jm990554g","volume":"43","author":"TM Willson","year":"2000","unstructured":"Willson, T.M., Brown, P.J., Sternbach, D.D. & Henke, B.R. The PPARs: from orphan receptors to drug discovery. J. Med. Chem. 43, 527\u2013550 (2000).","journal-title":"J. Med. Chem."},{"key":"BFnm4003_CR37","doi-asserted-by":"crossref","first-page":"E1369","DOI":"10.1073\/pnas.1202464109","volume":"109","author":"K Iwaisako","year":"2012","unstructured":"Iwaisako, K. et al. Protection from liver fibrosis by a peroxisome proliferator-activated receptor\u2013\u03b4 agonist. Proc. Natl. Acad. Sci. USA 109, E1369\u2013E1376 (2012).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnm4003_CR38","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1093\/hmg\/8.3.397","volume":"8","author":"G Schilling","year":"1999","unstructured":"Schilling, G. et al. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin. Hum. Mol. Genet. 8, 397\u2013407 (1999).","journal-title":"Hum. Mol. Genet."},{"key":"BFnm4003_CR39","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1038\/nature11556","volume":"490","author":"SC Landis","year":"2012","unstructured":"Landis, S.C. et al. A call for transparent reporting to optimize the predictive value of preclinical research. Nature 490, 187\u2013191 (2012).","journal-title":"Nature"},{"key":"BFnm4003_CR40","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1038\/507423a","volume":"507","author":"S Perrin","year":"2014","unstructured":"Perrin, S. Preclinical research: make mouse studies work. Nature 507, 423\u2013425 (2014).","journal-title":"Nature"},{"key":"BFnm4003_CR41","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1038\/nm.2332","volume":"17","author":"M Lu","year":"2011","unstructured":"Lu, M. et al. Brain PPAR-\u03b3 promotes obesity and is required for the insulin-sensitizing effect of thiazolidinediones. Nat. Med. 17, 618\u2013622 (2011).","journal-title":"Nat. Med."},{"key":"BFnm4003_CR42","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1016\/j.nbd.2011.08.016","volume":"45","author":"MC Chiang","year":"2012","unstructured":"Chiang, M.C., Chern, Y. & Huang, R.N. PPAR-\u03b3 rescue of the mitochondrial dysfunction in Huntington\u2019s disease. Neurobiol. Dis. 45, 322\u2013328 (2012).","journal-title":"Neurobiol. Dis."},{"key":"BFnm4003_CR43","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1111\/jnc.12190","volume":"125","author":"J Jin","year":"2013","unstructured":"Jin, J. et al. Neuroprotective effects of PPAR-\u03b3 agonist rosiglitazone in N171-82Q mouse model of Huntington\u2019s disease. J. Neurochem. 125, 410\u2013419 (2013).","journal-title":"J. Neurochem."},{"key":"BFnm4003_CR44","doi-asserted-by":"crossref","first-page":"52ra73","DOI":"10.1126\/scitranslmed.3001059","volume":"2","author":"B Zheng","year":"2010","unstructured":"Zheng, B. et al. PGC-1\u03b1, a potential therapeutic target for early intervention in Parkinson\u2019s disease. Sci. Transl. Med. 2, 52ra73 (2010).","journal-title":"Sci. Transl. Med."},{"key":"BFnm4003_CR45","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1016\/j.cell.2011.02.010","volume":"144","author":"JH Shin","year":"2011","unstructured":"Shin, J.H. et al. PARIS (ZNF746) repression of PGC-1\u03b1 contributes to neurodegeneration in Parkinson\u2019s disease. Cell 144, 689\u2013702 (2011).","journal-title":"Cell"},{"key":"BFnm4003_CR46","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1016\/j.cell.2014.03.012","volume":"157","author":"RM Evans","year":"2014","unstructured":"Evans, R.M. & Mangelsdorf, D.J. Nuclear receptors, RXR, and the big bang. Cell 157, 255\u2013266 (2014).","journal-title":"Cell"},{"key":"BFnm4003_CR47","doi-asserted-by":"crossref","first-page":"1503","DOI":"10.1126\/science.1217697","volume":"335","author":"PE Cramer","year":"2012","unstructured":"Cramer, P.E. et al. ApoE-directed therapeutics rapidly clear \u03b2-amyloid and reverse deficits in AD mouse models. Science 335, 1503\u20131506 (2012).","journal-title":"Science"},{"key":"BFnm4003_CR48","doi-asserted-by":"crossref","first-page":"924","DOI":"10.1126\/science.1235809","volume":"340","author":"NF Fitz","year":"2013","unstructured":"Fitz, N.F., Cronican, A.A., Lefterov, I. & Koldamova, R. Comment on \u201cApoE-directed therapeutics rapidly clear \u03b2-amyloid and reverse deficits in AD mouse models.\u201d. Science 340, 924 (2013).","journal-title":"Science"},{"key":"BFnm4003_CR49","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1093\/hmg\/ddg046","volume":"12","author":"S Gines","year":"2003","unstructured":"Gines, S. et al. Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington\u2019s disease knock-in mice. Hum. Mol. Genet. 12, 497\u2013508 (2003).","journal-title":"Hum. Mol. Genet."},{"key":"BFnm4003_CR50","doi-asserted-by":"crossref","first-page":"2363","DOI":"10.1074\/jbc.M806088200","volume":"284","author":"JE Young","year":"2009","unstructured":"Young, J.E., Martinez, R.A. & La Spada, A.R. Nutrient deprivation induces neuronal autophagy and implicates reduced insulin signaling in neuroprotective autophagy activation. J. Biol. Chem. 284, 2363\u20132373 (2009).","journal-title":"J. Biol. Chem."},{"key":"BFnm4003_CR51","doi-asserted-by":"crossref","first-page":"1987","DOI":"10.1523\/JNEUROSCI.4072-08.2009","volume":"29","author":"JE Young","year":"2009","unstructured":"Young, J.E. et al. Polyglutamine-expanded androgen receptor truncation fragments activate a Bax-dependent apoptotic cascade mediated by DP5 (Hrk). J. Neurosci. 29, 1987\u20131997 (2009).","journal-title":"J. Neurosci."},{"key":"BFnm4003_CR52","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1016\/j.nbd.2007.11.006","volume":"29","author":"J Nithianantharajah","year":"2008","unstructured":"Nithianantharajah, J., Barkus, C., Murphy, M. & Hannan, A.J. Gene-environment interactions modulating cognitive function and molecular correlates of synaptic plasticity in Huntington\u2019s disease transgenic mice. Neurobiol. Dis. 29, 490\u2013504 (2008).","journal-title":"Neurobiol. Dis."},{"key":"BFnm4003_CR53","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1016\/S0896-6273(04)00082-0","volume":"41","author":"BL Sopher","year":"2004","unstructured":"Sopher, B.L. et al. Androgen receptor YAC\u2013transgenic mice recapitulate SBMA motor neuronopathy and implicate VEGF164 in the motor neuron degeneration. Neuron 41, 687\u2013699 (2004).","journal-title":"Neuron"},{"key":"BFnm4003_CR54","doi-asserted-by":"crossref","first-page":"4897","DOI":"10.1523\/JNEUROSCI.22-12-04897.2002","volume":"22","author":"GA Garden","year":"2002","unstructured":"Garden, G.A. et al. Polyglutamine-expanded ataxin-7 promotes non\u2013cell-autonomous Purkinje cell degeneration and displays proteolytic cleavage in ataxic transgenic mice. J. Neurosci. 22, 4897\u20134905 (2002).","journal-title":"J. Neurosci."},{"key":"BFnm4003_CR55","doi-asserted-by":"crossref","first-page":"913","DOI":"10.1016\/S0896-6273(01)00422-6","volume":"31","author":"AR La Spada","year":"2001","unstructured":"La Spada, A.R. et al. Polyglutamine-expanded ataxin-7 antagonizes CRX function and induces cone-rod dystrophy in a mouse model of SCA7. Neuron 31, 913\u2013927 (2001).","journal-title":"Neuron"},{"key":"BFnm4003_CR56","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1373\/clinchem.2006.078873","volume":"53","author":"AJ Janssen","year":"2007","unstructured":"Janssen, A.J. et al. Spectrophotometric assay for complex I of the respiratory chain in tissue samples and cultured fibroblasts. Clin. Chem. 53, 729\u2013734 (2007).","journal-title":"Clin. Chem."},{"key":"BFnm4003_CR57","doi-asserted-by":"crossref","first-page":"16707","DOI":"10.1073\/pnas.0808488105","volume":"105","author":"L Aubry","year":"2008","unstructured":"Aubry, L. et al. Striatal progenitors derived from human ES cells mature into DARPP32 neurons in vitro and in quinolinic acid\u2013lesioned rats. Proc. Natl. Acad. Sci. USA 105, 16707\u201316712 (2008).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"issue":"2","key":"BFnm4003_CR58","doi-asserted-by":"crossref","first-page":"264","DOI":"10.1016\/j.stem.2012.04.027","volume":"11","author":"The\u00a0HD\u00a0iPSC\u00a0Consortium","year":"2012","unstructured":"HD iPSC Consortium. Induced pluripotent stem cells from patients with Huntington\u2019s disease show CAG-repeat expansion\u2013associated phenotypes. Cell Stem Cell 11, 264\u2013278 (2012).","journal-title":"Cell Stem Cell"}],"container-title":["Nature Medicine"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.nature.com\/articles\/nm.4003.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.nature.com\/articles\/nm.4003","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.nature.com\/articles\/nm.4003.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,18]],"date-time":"2023-05-18T18:54:26Z","timestamp":1684436066000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/nm.4003"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,12,7]]},"references-count":58,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2016,1]]}},"alternative-id":["BFnm4003"],"URL":"https:\/\/doi.org\/10.1038\/nm.4003","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.725995535.793512588","asserted-by":"object"}]},"ISSN":["1078-8956","1546-170X"],"issn-type":[{"value":"1078-8956","type":"print"},{"value":"1546-170X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,12,7]]},"assertion":[{"value":"30 June 2015","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 November 2015","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 December 2015","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The authors declare no competing financial interests.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}]}}