{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T02:17:51Z","timestamp":1791253071976,"version":"4.1.0"},"reference-count":76,"publisher":"Oxford University Press (OUP)","issue":"16","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2009,8,15]]},"DOI":"10.1093\/hmg\/ddp243","type":"journal-article","created":{"date-parts":[[2009,5,21]],"date-time":"2009-05-21T20:55:43Z","timestamp":1242939343000},"page":"3048-3065","source":"Crossref","is-referenced-by-count":213,"title":["Impaired PGC-1\u03b1 function in muscle in Huntington's disease"],"prefix":"10.1093","volume":"18","author":[{"given":"Rajnish K.","family":"Chaturvedi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Adhihetty","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shubha","family":"Shukla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Hennessy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noel","family":"Calingasan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lichuan","family":"Yang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anatoly","family":"Starkov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mahmoud","family":"Kiaei","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Milena","family":"Cannella","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jenny","family":"Sassone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Ciammola","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fernando","family":"Squitieri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. Flint","family":"Beal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2009,5,21]]},"reference":[{"key":"key\n\t\t\t\t20170522173433_DDP243C1","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1002\/1531-8249(200001)47:1<64::AID-ANA11>3.0.CO;2-S","article-title":"Higher sedentary energy expenditure in patients with Huntington's disease","volume":"47","author":"Pratley","year":"2000","journal-title":"Ann. Neurol."},{"key":"key\n\t\t\t\t20170522173433_DDP243C2","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1016\/j.expneurol.2007.12.026","article-title":"The metabolic profile of early Huntington's disease- a combined human and transgenic mouse study","volume":"210","author":"Goodman","year":"2008","journal-title":"Exp. Neurol."},{"key":"key\n\t\t\t\t20170522173433_DDP243C3","doi-asserted-by":"crossref","first-page":"1325","DOI":"10.1212\/01.WNL.0000031791.10922.CF","article-title":"Weight loss in early stage of Huntington's disease","volume":"59","author":"Djousse","year":"2002","journal-title":"Neurology"},{"key":"key\n\t\t\t\t20170522173433_DDP243C4","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1093\/hmg\/8.5.813","article-title":"Formation of polyglutamine inclusions in non-CNS tissue","volume":"8","author":"Sathasivam","year":"1999","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522173433_DDP243C5","doi-asserted-by":"crossref","first-page":"3092","DOI":"10.1111\/j.1460-9568.2004.03783.x","article-title":"Progressive abnormalities in skeletal muscle and neuromuscular junctions of transgenic mice expressing the Huntington's disease mutation","volume":"20","author":"Ribchester","year":"2004","journal-title":"Eur. J. Neurosci."},{"key":"key\n\t\t\t\t20170522173433_DDP243C6","doi-asserted-by":"crossref","first-page":"1161","DOI":"10.1001\/archneur.1992.00530350075022","article-title":"Serial changes of cerebral glucose metabolism and caudate size in persons at risk for Huntington's disease","volume":"49","author":"Grafton","year":"1992","journal-title":"Arch. Neurol."},{"key":"key\n\t\t\t\t20170522173433_DDP243C7","first-page":"1591","article-title":"Metabolic network abnormalities in early Huntington's disease: an [(18)F]FDG PET study","volume":"42","author":"Feigin","year":"2001","journal-title":"J. Nucl. Med."},{"issue":"Pt 6","key":"key\n\t\t\t\t20170522173433_DDP243C8","doi-asserted-by":"crossref","first-page":"2085","DOI":"10.1093\/brain\/119.6.2085","article-title":"Striatal glucose metabolism and dopamine D2 receptor binding in asymptomatic gene carriers and patients with Huntington's disease","volume":"119","author":"Antonini","year":"1996","journal-title":"Brain"},{"key":"key\n\t\t\t\t20170522173433_DDP243C9","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1007\/BF00870669","article-title":"Striatal glucose consumption in chorea-free subjects at risk of Huntington's disease","volume":"241","author":"Kuwert","year":"1993","journal-title":"J. Neurol."},{"key":"key\n\t\t\t\t20170522173433_DDP243C10","doi-asserted-by":"crossref","first-page":"2689","DOI":"10.1212\/WNL.43.12.2689","article-title":"Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy","volume":"43","author":"Jenkins","year":"1993","journal-title":"Neurology"},{"key":"key\n\t\t\t\t20170522173433_DDP243C11","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1002\/ana.410410206","article-title":"Energy metabolism defects in Huntington's disease and effects of coenzyme Q10","volume":"41","author":"Koroshetz","year":"1997","journal-title":"Ann. Neurol."},{"key":"key\n\t\t\t\t20170522173433_DDP243C12","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1002\/1531-8249(200007)48:1<72::AID-ANA11>3.0.CO;2-I","article-title":"Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropallidoluysian atrophy","volume":"48","author":"Lodi","year":"2000","journal-title":"Ann. Neurol."},{"key":"key\n\t\t\t\t20170522173433_DDP243C13","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1002\/mds.20373","article-title":"Mitochondrial impairment in patients and asymptomatic mutation carriers of Huntington's disease","volume":"20","author":"Saft","year":"2005","journal-title":"Mov. Disord."},{"key":"key\n\t\t\t\t20170522173433_DDP243C14","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1002\/ana.410410514","article-title":"Oxidative damage and metabolic dysfunction in Huntington's disease: selective vulnerability of the basal ganglia","volume":"41","author":"Browne","year":"1997","journal-title":"Ann. Neurol."},{"key":"key\n\t\t\t\t20170522173433_DDP243C15","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1002\/ana.410390317","article-title":"Mitochondrial defect in Huntington's disease caudate nucleus","volume":"39","author":"Gu","year":"1996","journal-title":"Ann. Neurol."},{"key":"key\n\t\t\t\t20170522173433_DDP243C16","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1002\/1531-8249(199901)45:1<25::AID-ART6>3.0.CO;2-E","article-title":"Biochemical abnormalities and excitotoxicity in Huntington's disease brain","volume":"45","author":"Tabrizi","year":"1999","journal-title":"Ann. Neurol."},{"key":"key\n\t\t\t\t20170522173433_DDP243C17","doi-asserted-by":"crossref","first-page":"2871","DOI":"10.1093\/hmg\/ddi319","article-title":"HD CAG repeat implicates a dominant property of huntingtin in mitochondrial energy metabolism","volume":"14","author":"Seong","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522173433_DDP243C18","doi-asserted-by":"crossref","first-page":"30773","DOI":"10.1074\/jbc.M504749200","article-title":"Mitochondrial respiration and ATP production are significantly impaired in striatal cells expressing mutant huntingtin","volume":"280","author":"Milakovic","year":"2005","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522173433_DDP243C19","doi-asserted-by":"crossref","first-page":"1147","DOI":"10.1111\/j.1471-4159.1993.tb03633.x","article-title":"Age-dependent striatal excitotoxic lesions produced by the endogenous mitochondrial inhibitor malonate","volume":"61","author":"Beal","year":"1993","journal-title":"J. Neurochem."},{"key":"key\n\t\t\t\t20170522173433_DDP243C20","doi-asserted-by":"crossref","first-page":"7105","DOI":"10.1073\/pnas.92.15.7105","article-title":"Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates","volume":"92","author":"Brouillet","year":"1995","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522173433_DDP243C21","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1017\/S0317167100032212","article-title":"3-Nitropropionic acid-exogenous animal neurotoxin and possible human striatal toxin","volume":"18","author":"Ludolph","year":"1991","journal-title":"Can. J. Neurol. Sci."},{"key":"key\n\t\t\t\t20170522173433_DDP243C22","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1111\/j.1471-4159.1993.tb05859.x","article-title":"Age-dependent vulnerability of the striatum to the mitochondrial toxin 3-nitropropionic acid","volume":"60","author":"Brouillet","year":"1993","journal-title":"J. Neurochem."},{"key":"key\n\t\t\t\t20170522173433_DDP243C23","doi-asserted-by":"crossref","first-page":"2783","DOI":"10.1523\/JNEUROSCI.0106-08.2008","article-title":"N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking","volume":"28","author":"Orr","year":"2008","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522173433_DDP243C24","doi-asserted-by":"crossref","first-page":"1407","DOI":"10.1093\/hmg\/ddh162","article-title":"Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release","volume":"13","author":"Choo","year":"2004","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522173433_DDP243C25","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1046\/j.1471-4159.2003.02009.x","article-title":"Inclusion formation in Huntington's disease R6\/2 mouse muscle cultures","volume":"87","author":"Orth","year":"2003","journal-title":"J. Neurochem."},{"key":"key\n\t\t\t\t20170522173433_DDP243C26","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1038\/nn884","article-title":"Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines","volume":"5","author":"Panov","year":"2002","journal-title":"Nat. Neurosci."},{"key":"key\n\t\t\t\t20170522173433_DDP243C27","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1016\/S0166-2236(00)01609-X","article-title":"Transcriptional dysregulation in Huntington's disease","volume":"23","author":"Cha","year":"2000","journal-title":"Trends Neurosci."},{"key":"key\n\t\t\t\t20170522173433_DDP243C28","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1016\/S0168-9525(03)00074-X","article-title":"Transcriptional abnormalities in Huntington disease","volume":"19","author":"Sugars","year":"2003","journal-title":"Trends Genet."},{"key":"key\n\t\t\t\t20170522173433_DDP243C29","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1016\/j.nbd.2005.11.001","article-title":"Decreased association of the transcription factor Sp1 with genes downregulated in Huntington's disease","volume":"22","author":"Chen-Plotkin","year":"2006","journal-title":"Neurobiol. Dis."},{"key":"key\n\t\t\t\t20170522173433_DDP243C30","doi-asserted-by":"crossref","first-page":"2238","DOI":"10.1126\/science.1072613","article-title":"Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease","volume":"296","author":"Dunah","year":"2002","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522173433_DDP243C31","doi-asserted-by":"crossref","first-page":"2423","DOI":"10.1126\/science.1056784","article-title":"Interference by huntingtin and atrophin-1 with cbp-mediated transcription leading to cellular toxicity","volume":"291","author":"Nucifora","year":"2001","journal-title":"Science"},{"key":"key\n\t\t\t\t20170522173433_DDP243C32","doi-asserted-by":"crossref","first-page":"6763","DOI":"10.1073\/pnas.100110097","article-title":"The Huntington's disease protein interacts with p53 and CREB-binding protein and represses transcription","volume":"97","author":"Steffan","year":"2000","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522173433_DDP243C33","doi-asserted-by":"crossref","first-page":"e101","DOI":"10.1371\/journal.pbio.0030101","article-title":"PGC-1alpha deficiency causes multi-system energy metabolic derangements: muscle dysfunction, abnormal weight control and hepatic steatosis","volume":"3","author":"Leone","year":"2005","journal-title":"PLoS Biol."},{"key":"key\n\t\t\t\t20170522173433_DDP243C34","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.cell.2004.09.013","article-title":"Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice","volume":"119","author":"Lin","year":"2004","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522173433_DDP243C35","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1210\/er.2002-0012","article-title":"Peroxisome proliferator-activated receptor-gamma coactivator 1 alpha (PGC-1 alpha): transcriptional coactivator and metabolic regulator","volume":"24","author":"Puigserver","year":"2003","journal-title":"Endocr. Rev."},{"key":"key\n\t\t\t\t20170522173433_DDP243C36","doi-asserted-by":"crossref","first-page":"829","DOI":"10.1016\/S0092-8674(00)81410-5","article-title":"A cold-inducible coactivator of nuclear receptors linked to adaptive thermogenesis","volume":"92","author":"Puigserver","year":"1998","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522173433_DDP243C37","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1016\/j.cmet.2005.05.004","article-title":"Metabolic control through the PGC-1 family of transcription coactivators","volume":"1","author":"Lin","year":"2005","journal-title":"Cell Metab."},{"key":"key\n\t\t\t\t20170522173433_DDP243C38","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1101\/gad.1177604","article-title":"Transcriptional regulatory circuits controlling mitochondrial biogenesis and function","volume":"18","author":"Kelly","year":"2004","journal-title":"Genes Dev."},{"key":"key\n\t\t\t\t20170522173433_DDP243C39","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.cell.2006.09.015","article-title":"Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration","volume":"127","author":"Cui","year":"2006","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522173433_DDP243C40","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/j.cmet.2006.10.004","article-title":"Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration","volume":"4","author":"Weydt","year":"2006","journal-title":"Cell Metab."},{"key":"key\n\t\t\t\t20170522173433_DDP243C41","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1038\/nature00904","article-title":"Transcriptional co-activator PGC-1 alpha drives the formation of slow-twitch muscle fibres","volume":"418","author":"Lin","year":"2002","journal-title":"Nature"},{"key":"key\n\t\t\t\t20170522173433_DDP243C42","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1002\/ana.20754","article-title":"Low stability of Huntington muscle mitochondria against Ca2+ in R6\/2 mice","volume":"59","author":"Gizatullina","year":"2006","journal-title":"Ann. Neurol."},{"key":"key\n\t\t\t\t20170522173433_DDP243C43","doi-asserted-by":"crossref","first-page":"1637","DOI":"10.1002\/mds.21550","article-title":"Myopathy as a first symptom of Huntington's disease in a Marathon runner","volume":"22","author":"Kosinski","year":"2007","journal-title":"Mov. Disord."},{"key":"key\n\t\t\t\t20170522173433_DDP243C44","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1016\/j.nbd.2006.09.016","article-title":"Cardiac dysfunction in the R6\/2 mouse model of Huntington's disease","volume":"25","author":"Mihm","year":"2007","journal-title":"Neurobiol. Dis."},{"key":"key\n\t\t\t\t20170522173433_DDP243C45","doi-asserted-by":"crossref","first-page":"1863","DOI":"10.1093\/hmg\/ddi192","article-title":"Gene expression in Huntington's disease skeletal muscle: a potential biomarker","volume":"14","author":"Strand","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522173433_DDP243C46","doi-asserted-by":"crossref","first-page":"1715","DOI":"10.1002\/mds.21540","article-title":"Clinical correlates of mitochondrial function in Huntington's disease muscle","volume":"22","author":"Turner","year":"2007","journal-title":"Mov. Disord."},{"key":"key\n\t\t\t\t20170522173433_DDP243C47","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1093\/hmg\/ddg046","article-title":"Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice","volume":"12","author":"Gines","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522173433_DDP243C48","doi-asserted-by":"crossref","first-page":"1060","DOI":"10.1016\/S0021-9258(19)42941-4","article-title":"Experimental depletion of creatine and phosphocreatine from skeletal muscle","volume":"249","author":"Fitch","year":"1974","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522173433_DDP243C49","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1042\/bj2320125","article-title":"Biochemical adaptation in the skeletal muscle of rats depleted of creatine with the substrate analogue beta-guanidinopropionic acid","volume":"232","author":"Shoubridge","year":"1985","journal-title":"Biochem. J."},{"key":"key\n\t\t\t\t20170522173433_DDP243C50","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1016\/0005-2728(96)00074-6","article-title":"Differential effects of creatine depletion on the regulation of enzyme activities and on creatine-stimulated mitochondrial respiration in skeletal muscle, heart, and brain","volume":"1276","author":"O'Gorman","year":"1996","journal-title":"Biochim. Biophys. Acta"},{"key":"key\n\t\t\t\t20170522173433_DDP243C51","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1046\/j.1365-201x.1998.00377.x","article-title":"Effect of chronic electrical stimulation and beta-GPA diet on GLUT4 protein concentration in rat skeletal muscle","volume":"163","author":"Yaspelkis","year":"1998","journal-title":"Acta Physiol. Scand."},{"key":"key\n\t\t\t\t20170522173433_DDP243C52","doi-asserted-by":"crossref","first-page":"15983","DOI":"10.1073\/pnas.252625599","article-title":"AMP kinase is required for mitochondrial biogenesis in skeletal muscle in response to chronic energy deprivation","volume":"99","author":"Zong","year":"2002","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522173433_DDP243C53","doi-asserted-by":"crossref","first-page":"12017","DOI":"10.1073\/pnas.0705070104","article-title":"AMP-activated protein kinase (AMPK) action in skeletal muscle via direct phosphorylation of PGC-1alpha","volume":"104","author":"Jager","year":"2007","journal-title":"Proc. Natl. Acad. Sci. USA"},{"issue":"Pt 3","key":"key\n\t\t\t\t20170522173433_DDP243C54","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1042\/bj3450437","article-title":"The regulation of AMP-activated protein kinase by phosphorylation","volume":"345","author":"Stein","year":"2000","journal-title":"Biochem. J."},{"key":"key\n\t\t\t\t20170522173433_DDP243C55","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1038\/ng1180","article-title":"PGC-1alpha-responsive genes involved in oxidative phosphorylation are coordinately downregulated in human diabetes","volume":"34","author":"Mootha","year":"2003","journal-title":"Nat. Genet."},{"key":"key\n\t\t\t\t20170522173433_DDP243C56","doi-asserted-by":"crossref","first-page":"15545","DOI":"10.1073\/pnas.0506580102","article-title":"Gene set enrichment analysis: a knowledge-based approach for interpreting genome-wide expression profiles","volume":"102","author":"Subramanian","year":"2005","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522173433_DDP243C57","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/j.cmet.2006.12.003","article-title":"The transcriptional coactivator PGC-1beta drives the formation of oxidative type IIX fibers in skeletal muscle","volume":"5","author":"Arany","year":"2007","journal-title":"Cell Metab."},{"key":"key\n\t\t\t\t20170522173433_DDP243C58","doi-asserted-by":"crossref","first-page":"6570","DOI":"10.1073\/pnas.0401401101","article-title":"Erralpha and Gabpa\/b specify PGC-1alpha-dependent oxidative phosphorylation gene expression that is altered in diabetic muscle","volume":"101","author":"Mootha","year":"2004","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522173433_DDP243C59","doi-asserted-by":"crossref","first-page":"26597","DOI":"10.1074\/jbc.M301850200","article-title":"Bioenergetic analysis of peroxisome proliferator-activated receptor gamma coactivators 1alpha and 1beta (PGC-1alpha and PGC-1beta) in muscle cells","volume":"278","author":"St-Pierre","year":"2003","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522173433_DDP243C60","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/S0092-8674(00)80611-X","article-title":"Mechanisms controlling mitochondrial biogenesis and respiration through the thermogenic coactivator PGC-1","volume":"98","author":"Wu","year":"1999","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522173433_DDP243C61","doi-asserted-by":"crossref","first-page":"568","DOI":"10.1161\/01.RES.0000141774.29937.e3","article-title":"Nuclear receptor signaling and cardiac energetics","volume":"95","author":"Huss","year":"2004","journal-title":"Circ. Res."},{"key":"key\n\t\t\t\t20170522173433_DDP243C62","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1038\/ng0398-231","article-title":"Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice","volume":"18","author":"Larsson","year":"1998","journal-title":"Nat. Genet."},{"key":"key\n\t\t\t\t20170522173433_DDP243C63","doi-asserted-by":"crossref","first-page":"2061","DOI":"10.1089\/ars.2006.8.2061","article-title":"Oxidative damage in Huntington's disease pathogenesis","volume":"8","author":"Browne","year":"2006","journal-title":"Antioxid. Redox Signal"},{"key":"key\n\t\t\t\t20170522173433_DDP243C64","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1212\/01.wnl.0000194318.74946.b6","article-title":"Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2\u2019dG","volume":"66","author":"Hersch","year":"2006","journal-title":"Neurology"},{"key":"key\n\t\t\t\t20170522173433_DDP243C65","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1016\/j.cell.2006.09.024","article-title":"Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators","volume":"127","author":"St-Pierre","year":"2006","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522173433_DDP243C66","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1016\/j.cmet.2005.03.002","article-title":"Transcriptional coactivator PGC-1 alpha controls the energy state and contractile function of cardiac muscle","volume":"1","author":"Arany","year":"2005","journal-title":"Cell Metab."},{"key":"key\n\t\t\t\t20170522173433_DDP243C67","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1101\/gad.1525107","article-title":"PGC-1alpha regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy","volume":"21","author":"Handschin","year":"2007","journal-title":"Genes Dev."},{"key":"key\n\t\t\t\t20170522173433_DDP243C68","doi-asserted-by":"crossref","first-page":"16260","DOI":"10.1073\/pnas.0607795103","article-title":"PGC-1alpha protects skeletal muscle from atrophy by suppressing FoxO3 action and atrophy-specific gene transcription","volume":"103","author":"Sandri","year":"2006","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522173433_DDP243C69","doi-asserted-by":"crossref","first-page":"1599","DOI":"10.1093\/hmg\/ddh175","article-title":"Nuclear-targeting of mutant huntingtin fragments produces Huntington's disease-like phenotypes in transgenic mice","volume":"13","author":"Schilling","year":"2004","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522173433_DDP243C70","doi-asserted-by":"crossref","first-page":"30014","DOI":"10.1074\/jbc.M704817200","article-title":"Skeletal muscle fiber-type switching, exercise intolerance, and myopathy in PGC-1alpha muscle-specific knock-out animals","volume":"282","author":"Handschin","year":"2007","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522173433_DDP243C71","doi-asserted-by":"crossref","first-page":"2068","DOI":"10.1038\/sj.cdd.4401967","article-title":"Increased apoptosis, Huntingtin inclusions and altered differentiation in muscle cell cultures from Huntington's disease subjects","volume":"13","author":"Ciammola","year":"2006","journal-title":"Cell Death Differ."},{"key":"key\n\t\t\t\t20170522173433_DDP243C72","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1136\/jcp.55.5.375","article-title":"Validation of a simple, rapid, and economical technique for distinguishing type 1 and 2 fibres in fixed and frozen skeletal muscle","volume":"55","author":"Behan","year":"2002","journal-title":"J. Clin. Pathol."},{"key":"key\n\t\t\t\t20170522173433_DDP243C73","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1016\/S1046-2023(02)00037-3","article-title":"Measurements of ATP in mammalian cells","volume":"26","author":"Manfredi","year":"2002","journal-title":"Methods"},{"key":"key\n\t\t\t\t20170522173433_DDP243C74","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1523\/JNEUROSCI.18-01-00156.1998","article-title":"Neuroprotective effects of creatine and cyclocreatine in animal models of Huntington's disease","volume":"18","author":"Matthews","year":"1998","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522173433_DDP243C75","doi-asserted-by":"crossref","first-page":"5623","DOI":"10.1073\/pnas.78.9.5623","article-title":"Isolation and characterization of human muscle cells","volume":"78","author":"Blau","year":"1981","journal-title":"Proc. Natl Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522173433_DDP243C76","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1007\/s00429-002-0233-1","article-title":"Targeting functional subtypes of spinal motoneurons and skeletal muscle fibers in vivo by intramuscular injection of adenoviral and adeno-associated viral vectors","volume":"205","author":"Martinov","year":"2002","journal-title":"Anat. Embryol."}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/18\/16\/3048\/17247332\/ddp243.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,10,5]],"date-time":"2021-10-05T22:26:58Z","timestamp":1633472818000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article-lookup\/doi\/10.1093\/hmg\/ddp243"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,5,21]]},"references-count":76,"journal-issue":{"issue":"16","published-online":{"date-parts":[[2009,5,21]]},"published-print":{"date-parts":[[2009,8,15]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddp243","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.1285962.753061","asserted-by":"object"}]},"ISSN":["1460-2083","0964-6906"],"issn-type":[{"value":"1460-2083","type":"electronic"},{"value":"0964-6906","type":"print"}],"subject":[],"published":{"date-parts":[[2009,5,21]]}}}