{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T22:41:54Z","timestamp":1792708914886,"version":"4.5.0"},"reference-count":157,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2009,10,1]],"date-time":"2009-10-01T00:00:00Z","timestamp":1254355200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Experimental Neurology"],"published-print":{"date-parts":[[2009,10]]},"DOI":"10.1016\/j.expneurol.2009.05.012","type":"journal-article","created":{"date-parts":[[2009,5,20]],"date-time":"2009-05-20T08:50:37Z","timestamp":1242809437000},"page":"385-397","source":"Crossref","is-referenced-by-count":153,"title":["Huntington's disease: The current state of research with peripheral tissues"],"prefix":"10.1016","volume":"219","author":[{"given":"Jenny","family":"Sassone","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clarissa","family":"Colciago","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuliana","family":"Cislaghi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincenzo","family":"Silani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Ciammola","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.expneurol.2009.05.012_bib1","doi-asserted-by":"crossref","first-page":"856","DOI":"10.1038\/39885","article-title":"Anterograde transport of brain-derived neurotrophic factor and its role in the brain","volume":"389","author":"Altar","year":"1997","journal-title":"Nature"},{"key":"10.1016\/j.expneurol.2009.05.012_bib2","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1093\/brain\/awm312","article-title":"Chromosomal profiles of gene expression in Huntington's disease","volume":"131","author":"Anderson","year":"2008","journal-title":"Brain"},{"key":"10.1016\/j.expneurol.2009.05.012_bib3","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1006\/nbdi.2002.0562","article-title":"Huntington's disease of the endocrine pancreas: insulin deficiency and diabetes mellitus due to impaired insulin gene expression","volume":"11","author":"Andreassen","year":"2002","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.expneurol.2009.05.012_bib4","doi-asserted-by":"crossref","first-page":"726","DOI":"10.1136\/jnnp.72.6.726","article-title":"Autonomic nervous system function in Huntington's disease","volume":"72","author":"Andrich","year":"2002","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"10.1016\/j.expneurol.2009.05.012_bib5","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1002\/ana.410430321","article-title":"Complex I defect in muscle from patients with Huntington's disease","volume":"43","author":"Arenas","year":"1998","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib6","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1038\/nature02998","article-title":"Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death","volume":"431","author":"Arrasate","year":"2004","journal-title":"Nature"},{"key":"10.1016\/j.expneurol.2009.05.012_bib7","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1515\/REVNEURO.2007.18.3-4.223","article-title":"Hypothalamic dysfunction and neuroendocrine and metabolic alterations in Huntington's disease: clinical consequences and therapeutic implications","volume":"18","author":"Aziz","year":"2007","journal-title":"Rev. Neurosci."},{"key":"10.1016\/j.expneurol.2009.05.012_bib8","doi-asserted-by":"crossref","first-page":"1506","DOI":"10.1212\/01.wnl.0000334276.09729.0e","article-title":"Weight loss in Huntington disease increases with higher CAG repeat number","volume":"71","author":"Aziz","year":"2008","journal-title":"Neurology"},{"key":"10.1016\/j.expneurol.2009.05.012_bib9","doi-asserted-by":"crossref","first-page":"1223","DOI":"10.1210\/jc.2008-2543","article-title":"Increased hypothalamic\u2013pituitary\u2013adrenal axis activity in Huntington's disease","volume":"94","author":"Aziz","year":"2009","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"10.1016\/j.expneurol.2009.05.012_bib10","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1007\/s00418-008-0393-z","article-title":"Islet beta-cell area and hormone expression are unaltered in Huntington's disease.","volume":"129","author":"Bacos","year":"2008","journal-title":"Histochem. Cell Biol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib11","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1111\/j.1468-1331.2008.02173.x","article-title":"Cardiovagal modulation upon postural change is altered in Huntington's disease","volume":"15","author":"B\u00e4r","year":"2008","journal-title":"Eur. J. Neurol."},{"issue":"292","key":"10.1016\/j.expneurol.2009.05.012_bib12","article-title":"Immunological studies in Huntington's disease","volume":"4","author":"Barkley","year":"1978","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib13","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1016\/0008-8749(77)90214-3","article-title":"Cellular immune responses in Huntington's disease (H.D.). Detection of H.D. and multiple sclerosis (M.S.) brain antigenicity by H.D. but not M.S. lymphocytes","volume":"32","author":"Barkley","year":"1977","journal-title":"Cell. Immunol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib14","doi-asserted-by":"crossref","first-page":"1642","DOI":"10.1016\/S0140-6736(03)13304-1","article-title":"Huntingtin aggregation and toxicity in Huntington's disease","volume":"361","author":"Bates","year":"2003","journal-title":"Lancet"},{"key":"10.1016\/j.expneurol.2009.05.012_bib15","doi-asserted-by":"crossref","first-page":"1552","DOI":"10.1126\/science.292.5521.1552","article-title":"Impairment of the ubiquitin\u2013proteasome system by protein aggregation","volume":"292","author":"Bence","year":"2001","journal-title":"Science"},{"key":"10.1016\/j.expneurol.2009.05.012_bib16","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1016\/j.neuroscience.2007.03.010","article-title":"Glutamate receptor abnormalities in the YAC128 transgenic mouse model of Huntington's disease","volume":"147","author":"Benn","year":"2007","journal-title":"Neuroscience"},{"key":"10.1016\/j.expneurol.2009.05.012_bib17","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1007\/BF00286586","article-title":"The current state of research with peripheral tissues in Huntington disease","volume":"66","author":"Beverstock","year":"1984","journal-title":"Hum. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib18","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1093\/hmg\/ddi053","article-title":"The R6\/2 transgenic mouse model of Huntington's disease develops diabetes due to deficient beta-cell mass and exocytosis","volume":"14","author":"Bj\u00f6rkqvist","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib19","doi-asserted-by":"crossref","first-page":"1713","DOI":"10.1093\/hmg\/ddl094","article-title":"Progressive alterations in the hypothalamic\u2013pituitary\u2013adrenal axis in the R6\/2 transgenic mouse model of Huntington's disease","volume":"15","author":"Bj\u00f6rkqvist","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib20","doi-asserted-by":"crossref","first-page":"1869","DOI":"10.1084\/jem.20080178","article-title":"A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease","volume":"205","author":"Bj\u00f6rkqvist","year":"2008","journal-title":"J. Exp. Med."},{"key":"10.1016\/j.expneurol.2009.05.012_bib21","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1016\/j.brainresrev.2008.04.001","article-title":"Magnetic resonance imaging as an approach towards identifying neuropathological biomarkers for Huntington's disease.","volume":"58","author":"Bohanna","year":"2008","journal-title":"Brain Res. Rev."},{"key":"10.1016\/j.expneurol.2009.05.012_bib22","doi-asserted-by":"crossref","first-page":"11023","DOI":"10.1073\/pnas.0504921102","article-title":"Genome-wide expression profiling of human blood reveals biomarkers for Huntington's disease","volume":"102","author":"Borovecki","year":"2005","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.expneurol.2009.05.012_bib23","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1038\/416507a","article-title":"Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases","volume":"416","author":"Bucciantini","year":"2002","journal-title":"Nature"},{"key":"10.1016\/j.expneurol.2009.05.012_bib24","doi-asserted-by":"crossref","first-page":"1534","DOI":"10.1007\/s00415-008-0964-x","article-title":"Use of hand-held dynamometry in the evaluation of lower limb muscle strength in people with Huntington's disease","volume":"255","author":"Busse","year":"2008","journal-title":"J. Neurol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib25","doi-asserted-by":"crossref","first-page":"3248","DOI":"10.1523\/JNEUROSCI.19-08-03248.1999","article-title":"Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation","volume":"19","author":"Carter","year":"1999","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2009.05.012_bib26","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":"10.1016\/j.expneurol.2009.05.012_bib27","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1016\/j.pneurobio.2007.03.004","article-title":"Transcriptional signatures in Huntington's disease","volume":"83","author":"Cha","year":"2007","journal-title":"Prog. Neurobiol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib28","doi-asserted-by":"crossref","first-page":"6480","DOI":"10.1073\/pnas.95.11.6480","article-title":"Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human Huntington disease gene","volume":"95","author":"Cha","year":"1998","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.expneurol.2009.05.012_bib29","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/j.bbrc.2007.05.093","article-title":"Increased oxidative damage and mitochondrial abnormalities in the peripheral blood of Huntington's disease patients","volume":"359","author":"Chen","year":"2007","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.expneurol.2009.05.012_bib30","doi-asserted-by":"crossref","first-page":"153","DOI":"10.5694\/j.1326-5377.1982.tb132224.x","article-title":"Causes of death in Huntington's disease","volume":"1","author":"Chiu","year":"1982","journal-title":"Med. J. Aust."},{"key":"10.1016\/j.expneurol.2009.05.012_bib31","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":"10.1016\/j.expneurol.2009.05.012_bib32","doi-asserted-by":"crossref","first-page":"574","DOI":"10.1002\/ajmg.b.30501","article-title":"Low brain-derived neurotrophic factor (BDNF) levels in serum of Huntington's disease patients","volume":"144B","author":"Ciammola","year":"2007","journal-title":"Am. J. Med. Genet. B. Neuropsychiatr. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib33","doi-asserted-by":"crossref","first-page":"1478","DOI":"10.1111\/j.1460-9568.2005.03985.x","article-title":"Akt is altered in an animal model of Huntington's disease and in patients","volume":"21","author":"Colin","year":"2005","journal-title":"Eur. J. Neurosci."},{"key":"10.1016\/j.expneurol.2009.05.012_bib34","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.mcn.2008.04.012","article-title":"Blood level of brain-derived neurotrophic factor mRNA is progressively reduced in rodent models of Huntington's disease: restoration by the neuroprotective compound CEP-1347","volume":"39","author":"Conforti","year":"2008","journal-title":"Mol. Cell. Neurosci."},{"key":"10.1016\/j.expneurol.2009.05.012_bib35","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":"10.1016\/j.expneurol.2009.05.012_bib36","doi-asserted-by":"crossref","first-page":"2833","DOI":"10.1021\/pr0700753","article-title":"Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates","volume":"6","author":"Dalrymple","year":"2007","journal-title":"J. Proteome Res."},{"key":"10.1016\/j.expneurol.2009.05.012_bib37","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1016\/S0092-8674(00)80513-9","article-title":"Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation","volume":"90","author":"Davies","year":"1997","journal-title":"Cell"},{"key":"10.1016\/j.expneurol.2009.05.012_bib38","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1126\/science.277.5334.1990","article-title":"Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain","volume":"277","author":"DiFiglia","year":"1997","journal-title":"Science"},{"key":"10.1016\/j.expneurol.2009.05.012_bib39","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":"Djouss\u00e9","year":"2002","journal-title":"Neurology"},{"key":"10.1016\/j.expneurol.2009.05.012_bib40","doi-asserted-by":"crossref","first-page":"2446","DOI":"10.1210\/en.2002-0113","article-title":"Reversal of behavioral and metabolic abnormalities, and insulin resistance syndrome, by dietary restriction in mice deficient in brain-derived neurotrophic factor","volume":"144","author":"Duan","year":"2003","journal-title":"Endocrinology"},{"key":"10.1016\/j.expneurol.2009.05.012_bib41","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1093\/hmg\/10.2.145","article-title":"Abnormalities in the functioning of adipocytes from R6\/2 mice that are transgenic for the Huntington's disease mutation","volume":"10","author":"Fain","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib42","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1111\/j.1399-0004.1985.tb00185.x","article-title":"Diabetes mellitus in Huntington disease","volume":"27","author":"Farrer","year":"1985","journal-title":"Clin. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib43","doi-asserted-by":"crossref","first-page":"1335","DOI":"10.1093\/ajcn\/81.6.1335","article-title":"Energy balance in early-stage Huntington disease","volume":"81","author":"Gaba","year":"2005","journal-title":"Am. J. Clin. Nutr."},{"issue":"288","key":"10.1016\/j.expneurol.2009.05.012_bib44","article-title":"Huntingtin gene evolution in Chordata and its peculiar features in the ascidian Ciona genus","volume":"8","author":"Gissi","year":"2006","journal-title":"BMC Genomics"},{"key":"10.1016\/j.expneurol.2009.05.012_bib45","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":"10.1016\/j.expneurol.2009.05.012_bib46","doi-asserted-by":"crossref","first-page":"S74","DOI":"10.1212\/01.wnl.0000192103.24796.42","article-title":"Common structure and toxic function of amyloid oligomers implies a common mechanism of pathogenesis","volume":"66","author":"Glabe","year":"2006","journal-title":"Neurology"},{"key":"10.1016\/j.expneurol.2009.05.012_bib47","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1016\/S0306-4522(00)00008-7","article-title":"The pattern of neurodegeneration in Huntington's disease: a comparative study of cannabinoid, dopamine, adenosine and GABA(A) receptor alterations in the human basal ganglia in Huntington's disease","volume":"97","author":"Glass","year":"2000","journal-title":"Neuroscience"},{"key":"10.1016\/j.expneurol.2009.05.012_bib48","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":"10.1016\/j.expneurol.2009.05.012_bib49","first-page":"259","article-title":"Targeting disease, not disease targets: innovative approaches in tackling neurodegenerative disorders","volume":"10","author":"Griffioen","year":"2007","journal-title":"IDrugs"},{"key":"10.1016\/j.expneurol.2009.05.012_bib50","doi-asserted-by":"crossref","first-page":"2522","DOI":"10.1523\/JNEUROSCI.19-07-02522.1999","article-title":"Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology","volume":"19","author":"Gutekunst","year":"1999","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2009.05.012_bib51","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1038\/nrm2101","article-title":"Soluble protein oligomers in neurodegeneration: lessons from the Alzheimer's amyloid beta-peptide","volume":"8","author":"Haass","year":"2007","journal-title":"Nat. Rev., Mol. Cell Biol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib52","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1136\/jnnp.2003.017822","article-title":"Rate and correlates of weight change in Huntington's disease","volume":"75","author":"Hamilton","year":"2004","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"10.1016\/j.expneurol.2009.05.012_bib53","doi-asserted-by":"crossref","first-page":"1387","DOI":"10.1038\/nn1997","article-title":"Microglia: active sensor and versatile effector cells in the normal and pathologic brain","volume":"10","author":"Hanisch","year":"2007","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.expneurol.2009.05.012_bib54","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes","volume":"72","year":"1993","journal-title":"Cell"},{"key":"10.1016\/j.expneurol.2009.05.012_bib55","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1016\/0006-3223(91)90007-9","article-title":"The limbic\u2013hypothalamic\u2013pituitary\u2013adrenal axis in Huntington's disease","volume":"30","author":"Heuser","year":"1991","journal-title":"Biol. Psychiatry"},{"key":"10.1016\/j.expneurol.2009.05.012_bib56","doi-asserted-by":"crossref","first-page":"649","DOI":"10.2337\/diabetes.48.3.649","article-title":"Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes","volume":"48","author":"Hurlbert","year":"1999","journal-title":"Diabetes"},{"key":"10.1016\/j.expneurol.2009.05.012_bib57","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1111\/j.1365-2826.2007.01629.x","article-title":"Impaired glucose tolerance in the R6\/1 transgenic mouse model of Huntington's disease","volume":"20","author":"Josefsen","year":"2008","journal-title":"J. Neuroendocrinol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib58","doi-asserted-by":"crossref","first-page":"221","DOI":"10.2174\/156652408784221298","article-title":"Molecular genetics and biomarkers of polyglutamine diseases","volume":"8","author":"Katsuno","year":"2008","journal-title":"Curr. Mol. Med."},{"key":"10.1016\/j.expneurol.2009.05.012_bib59","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1385\/NMM:1:3:183","article-title":"Cytochrome C and caspase-9 expression in Huntington's disease","volume":"1","author":"Kiechle","year":"2002","journal-title":"Neuromol. Med."},{"key":"10.1016\/j.expneurol.2009.05.012_bib60","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1146\/annurev.cellbio.15.1.1","article-title":"Vacuolar import of proteins and organelles from the cytoplasm","volume":"15","author":"Klionsky","year":"1999","journal-title":"Annu. Rev. Cell Dev. Biol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib61","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1242\/jcs.01620","article-title":"The molecular machinery of autophagy: unanswered questions","volume":"118","author":"Klionsky","year":"2005","journal-title":"J. Cell. Sci."},{"key":"10.1016\/j.expneurol.2009.05.012_bib62","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1111\/j.1468-1331.2004.00894.x","article-title":"Early sympathetic hyperactivity in Huntington's disease","volume":"11","author":"Kobal","year":"2004","journal-title":"Eur. J. Neurol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib63","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":"10.1016\/j.expneurol.2009.05.012_bib64","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":"10.1016\/j.expneurol.2009.05.012_bib65","doi-asserted-by":"crossref","first-page":"886","DOI":"10.1038\/nrc1738","article-title":"Lysosomes and autophagy in cell death control","volume":"5","author":"Kroemer","year":"2005","journal-title":"Nat. Rev., Cancer"},{"key":"10.1016\/j.expneurol.2009.05.012_bib66","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1002\/1531-8249(199912)46:6<842::AID-ANA6>3.0.CO;2-O","article-title":"Huntington aggregates may not predict neuronal death in Huntington's disease","volume":"46","author":"Kuemmerle","year":"1999","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib67","doi-asserted-by":"crossref","first-page":"878","DOI":"10.1001\/archneur.1988.00520320068017","article-title":"Conditions associated with Huntington's disease at death. A case-control study","volume":"45","author":"Lanska","year":"1988","journal-title":"Arch. Neurol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib68","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1212\/WNL.38.5.769","article-title":"Huntington's disease mortality in the United States","volume":"38","author":"Lanska","year":"1988","journal-title":"Neurology"},{"key":"10.1016\/j.expneurol.2009.05.012_bib69","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/0022-510X(95)00114-H","article-title":"Serum dehydroepiandrosterone and cortisol measurements in Huntington's chorea","volume":"132","author":"Leblhuber","year":"1995","journal-title":"J. Neurol. Sci."},{"key":"10.1016\/j.expneurol.2009.05.012_bib70","doi-asserted-by":"crossref","first-page":"632","DOI":"10.1056\/NEJM197803162981119","article-title":"Skin fibroblasts in Huntington's disease","volume":"298","author":"Leonardi","year":"1978","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.expneurol.2009.05.012_bib71","doi-asserted-by":"crossref","first-page":"2851","DOI":"10.1093\/brain\/awn212","article-title":"Plasma 24S-hydroxycholesterol and caudate MRI in pre-manifest and early Huntington's disease","volume":"131","author":"Leoni","year":"2008","journal-title":"Brain"},{"key":"10.1016\/j.expneurol.2009.05.012_bib72","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1093\/hmg\/7.5.777","article-title":"Aggregation of N-terminal huntingtin is dependent on the length of its glutamine repeats","volume":"7","author":"Li","year":"1998","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib73","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":"10.1016\/j.expneurol.2009.05.012_bib74","doi-asserted-by":"crossref","first-page":"1355","DOI":"10.1093\/hmg\/7.9.1355","article-title":"A cellular model that recapitulates major pathogenic steps of Huntington's disease","volume":"7","author":"Lunkes","year":"1998","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib75","doi-asserted-by":"crossref","first-page":"1911","DOI":"10.1093\/hmg\/11.17.1911","article-title":"Dysregulation of gene expression in the R6\/2 model of polyglutamine disease: parallel changes in muscle and brain","volume":"11","author":"Luthi-Carter","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib76","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1002\/ajmg.b.30223","article-title":"Adenosine A2A receptor dysfunction correlates with age at onset anticipation in blood platelets of subjects with Huntington's disease","volume":"139B","author":"Maglione","year":"2005","journal-title":"Am. J. Med. Genet. B. Neuropsychiatr. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib77","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1016\/j.mad.2005.09.011","article-title":"Huntingtin fragmentation and increased caspase 3, 8 and 9 activities in lymphoblasts with heterozygous and homozygous Huntington's disease mutation","volume":"127","author":"Maglione","year":"2006","journal-title":"Mech. Ageing Dev."},{"key":"10.1016\/j.expneurol.2009.05.012_bib78","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.neulet.2005.09.037","article-title":"The platelet maximum number of A2A-receptor binding sites (Bmax) linearly correlates with age at onset and CAG repeat expansion in Huntington's disease patients with predominant chorea","volume":"393","author":"Maglione","year":"2006","journal-title":"Neurosci. Lett."},{"key":"10.1016\/j.expneurol.2009.05.012_bib79","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1038\/ng0298-150","article-title":"Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates","volume":"18","author":"Martindale","year":"1998","journal-title":"Nat. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib80","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1016\/S1474-4422(07)70076-5","article-title":"Autophagy and neurodegeneration: when the cleaning crew goes on strike","volume":"6","author":"Martinez-Vicente","year":"2007","journal-title":"Lancet Neurol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib81","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1016\/j.cell.2006.10.023","article-title":"PGC-1alpha, a new therapeutic target in Huntington's disease?","volume":"127","author":"McGill","year":"2006","journal-title":"Cell"},{"key":"10.1016\/j.expneurol.2009.05.012_bib82","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1136\/jmg.19.3.168","article-title":"Sensitivity to ionosing radiation of lymphocytes from Huntington's chorea patients compared to controls","volume":"19","author":"McGovern","year":"1982","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib83","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1002\/ana.410010504","article-title":"Huntington's disease\u2014growth of fibroblast cultures in lipid-deficient medium: a preliminary report","volume":"1","author":"Menkes","year":"1977","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib84","doi-asserted-by":"crossref","first-page":"856","DOI":"10.1056\/NEJM197304192881624","article-title":"Fibroblast cultures in Huntington's disease","volume":"288","author":"Menkes","year":"1973","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.expneurol.2009.05.012_bib85","first-page":"64","article-title":"Huntington's disease: growth and fatty acid metabolism of fibroblast cultures in lipid-deficient medium","volume":"101","author":"Menkes","year":"1976","journal-title":"Trans. Am. Neurol. Assoc."},{"key":"10.1016\/j.expneurol.2009.05.012_bib86","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":"10.1016\/j.expneurol.2009.05.012_bib87","doi-asserted-by":"crossref","first-page":"e647","DOI":"10.1371\/journal.pone.0000647","article-title":"Early energy deficit in Huntington disease: identification of a plasma biomarker traceable during disease progression","volume":"2","author":"Mochel","year":"2007","journal-title":"PLoS ONE"},{"key":"10.1016\/j.expneurol.2009.05.012_bib88","doi-asserted-by":"crossref","first-page":"10417","DOI":"10.1073\/pnas.152161099","article-title":"The threshold for polyglutamine-expansion protein aggregation and cellular toxicity is dynamic and influenced by aging in Caenorhabditis elegans","volume":"99","author":"Morley","year":"2002","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.expneurol.2009.05.012_bib89","doi-asserted-by":"crossref","first-page":"1090","DOI":"10.1111\/j.1471-4159.2006.03998.x","article-title":"Genotype-dependent priming to self- and xeno-cannibalism in heterozygous and homozygous lymphoblasts from patients with Huntington's disease","volume":"98","author":"Mormone","year":"2006","journal-title":"J. Neurochem."},{"key":"10.1016\/j.expneurol.2009.05.012_bib90","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1038\/382536a0","article-title":"A phosphatidylinositol-3-OH kinase family member regulating longevity and diapause in Caenorhabditis elegans","volume":"382","author":"Morris","year":"1996","journal-title":"Nature"},{"key":"10.1016\/j.expneurol.2009.05.012_bib91","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1038\/nrn1587","article-title":"Modulation of neurodegeneration by molecular chaperones","volume":"6","author":"Muchowski","year":"2005","journal-title":"Nat. Rev., Neurosci."},{"key":"10.1016\/j.expneurol.2009.05.012_bib92","doi-asserted-by":"crossref","first-page":"10887","DOI":"10.1073\/pnas.0409283102","article-title":"Formation of morphologically similar globular aggregates from diverse aggregation-prone proteins in mammalian cells","volume":"102","author":"Mukai","year":"2005","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.expneurol.2009.05.012_bib93","series-title":"Genetic Instabilities and Hereditary Neurological Diseases","first-page":"301","article-title":"Huntington's disease","author":"Myers","year":"1998"},{"key":"10.1016\/j.expneurol.2009.05.012_bib94","doi-asserted-by":"crossref","first-page":"1325","DOI":"10.1097\/01.wnr.0000127073.66692.8f","article-title":"Autophagosome-like vacuole formation in Huntington's disease lymphoblasts","volume":"15","author":"Nagata","year":"2004","journal-title":"NeuroReport"},{"key":"10.1016\/j.expneurol.2009.05.012_bib95","doi-asserted-by":"crossref","first-page":"47","DOI":"10.2174\/092986708783330692","article-title":"Aggresome formation and neurodegenerative diseases: therapeutic implications","volume":"15","author":"Olzmann","year":"2008","journal-title":"Curr. Med. Chem."},{"key":"10.1016\/j.expneurol.2009.05.012_bib96","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1016\/j.compbiolchem.2007.11.001","article-title":"Molecular dynamics simulation study on the structural stabilities of polyglutamine peptides","volume":"32","author":"Ogawa","year":"2008","journal-title":"Comput. Biol. Chem."},{"key":"10.1016\/j.expneurol.2009.05.012_bib97","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":"10.1016\/j.expneurol.2009.05.012_bib98","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":"10.1016\/j.expneurol.2009.05.012_bib99","doi-asserted-by":"crossref","first-page":"877","DOI":"10.1016\/j.cell.2008.07.041","article-title":"Disease-specific induced pluripotent stem cells","volume":"134","author":"Park","year":"2008","journal-title":"Cell"},{"key":"10.1016\/j.expneurol.2009.05.012_bib100","doi-asserted-by":"crossref","first-page":"2743","DOI":"10.1161\/CIRCULATIONAHA.107.750232","article-title":"Cardiomyocyte expression of a polyglutamine preamyloid oligomer causes heart failure","volume":"117","author":"Pattison","year":"2008","journal-title":"Circulation"},{"key":"10.1016\/j.expneurol.2009.05.012_bib101","doi-asserted-by":"crossref","first-page":"1638","DOI":"10.1212\/01.wnl.0000222734.56412.17","article-title":"Microglial activation correlates with severity in Huntington disease: a clinical and PET study","volume":"66","author":"Pavese","year":"2006","journal-title":"Neurology"},{"key":"10.1016\/j.expneurol.2009.05.012_bib102","doi-asserted-by":"crossref","first-page":"961","DOI":"10.1111\/j.1460-9568.2006.04985.x","article-title":"Hypothalamic-endocrine aspects in Huntington's disease","volume":"24","author":"Peters\u00e9n","year":"2006","journal-title":"Eur. J. Neurosci."},{"key":"10.1016\/j.expneurol.2009.05.012_bib103","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1093\/hmg\/ddi004","article-title":"Orexin loss in Huntington's disease","volume":"14","author":"Peters\u00e9n","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib104","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1002\/ana.410080408","article-title":"Membrane studies in Huntington's disease: steady-state fluorescence studies of intact erythrocytes","volume":"8","author":"Pettegrew","year":"1980","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib105","doi-asserted-by":"crossref","first-page":"1966","DOI":"10.1111\/j.1471-4159.1981.tb10822.x","article-title":"Membrane studies in Huntington's disease: steady state and time-dependent fluorescence spectroscopy of intact lymphocytes","volume":"36","author":"Pettegrew","year":"1981","journal-title":"J. Neurochem."},{"key":"10.1016\/j.expneurol.2009.05.012_bib106","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1093\/hmg\/ddn428","article-title":"Adipose tissue dysfunction tracks disease progression in two Huntington's disease mouse models","volume":"18","author":"Phan","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib107","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1159\/000212174","article-title":"Abnormal glucose tolerance and arginine tolerance tests in Huntington's disease","volume":"23","author":"Podolsky","year":"1977","journal-title":"Gerontology"},{"key":"10.1016\/j.expneurol.2009.05.012_bib108","doi-asserted-by":"crossref","first-page":"1356","DOI":"10.1016\/S0140-6736(72)91092-6","article-title":"Increased frequency of diabetes mellitus in patients with Huntington's chorea","volume":"1","author":"Podolsky","year":"1972","journal-title":"Lancet"},{"key":"10.1016\/j.expneurol.2009.05.012_bib109","doi-asserted-by":"crossref","first-page":"41032","DOI":"10.1074\/jbc.M205809200","article-title":"Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization","volume":"277","author":"Poirier","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.expneurol.2009.05.012_bib110","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1530\/eje.0.1510451","article-title":"Circulating and cerebrospinal fluid ghrelin and leptin: potential role in altered body weight in Huntington's disease","volume":"151","author":"Popovic","year":"2004","journal-title":"Eur. J. Endocrinol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib111","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.nbd.2007.04.008","article-title":"Normal platelet mitochondrial complex I activity in Huntington's disease","volume":"27","author":"Powers","year":"2007","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.expneurol.2009.05.012_bib112","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":"10.1016\/j.expneurol.2009.05.012_bib113","doi-asserted-by":"crossref","first-page":"609","DOI":"10.5694\/j.1326-5377.1980.tb135166.x","article-title":"Presymptomatic detection of Huntington's disease","volume":"1","author":"Propert","year":"1980","journal-title":"Med. J. Aust."},{"key":"10.1016\/j.expneurol.2009.05.012_bib114","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":"10.1016\/j.expneurol.2009.05.012_bib115","doi-asserted-by":"crossref","first-page":"1107","DOI":"10.1093\/hmg\/11.9.1107","article-title":"Aggregate-prone proteins with polyglutamine and polyalanine expansions are degraded by autophagy","volume":"11","author":"Ravikumar","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib116","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1038\/ng1362","article-title":"Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease","volume":"36","author":"Ravikumar","year":"2004","journal-title":"Nat. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib117","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":"10.1016\/j.expneurol.2009.05.012_bib118","doi-asserted-by":"crossref","first-page":"510","DOI":"10.1007\/s00109-004-0552-1","article-title":"Neurodegenerative disorders associated with diabetes mellitus","volume":"82","author":"Ristow","year":"2004","journal-title":"J. Mol. Med."},{"key":"10.1016\/j.expneurol.2009.05.012_bib119","doi-asserted-by":"crossref","first-page":"14424","DOI":"10.1073\/pnas.0703652104","article-title":"Analysis of potential transcriptomic biomarkers for Huntington's disease in peripheral blood","volume":"104","author":"Runne","year":"2007","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.expneurol.2009.05.012_bib120","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":"10.1016\/j.expneurol.2009.05.012_bib121","doi-asserted-by":"crossref","first-page":"407","DOI":"10.5694\/j.1326-5377.1981.tb135681.x","article-title":"Body weight and dietary factors in Huntington's disease patients compared with matched controls","volume":"1","author":"Sanberg","year":"1981","journal-title":"Med. J. Aust."},{"key":"10.1016\/j.expneurol.2009.05.012_bib122","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1016\/S0896-6273(00)80716-3","article-title":"Caspase-8 is required for cell death induced by expanded polyglutamine repeats","volume":"22","author":"S\u00e1nchez","year":"1999","journal-title":"Neuron"},{"key":"10.1016\/j.expneurol.2009.05.012_bib123","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1002\/ana.410420411","article-title":"Huntingtin localization in brains of normal and Huntington's disease patients","volume":"42","author":"Sapp","year":"1997","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib124","doi-asserted-by":"crossref","first-page":"4263","DOI":"10.1111\/j.1742-4658.2008.06562.x","article-title":"Huntington's disease: degradation of mutant huntingtin by autophagy","volume":"275","author":"Sarkar","year":"2008","journal-title":"FEBS J."},{"key":"10.1016\/j.expneurol.2009.05.012_bib125","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":"10.1016\/j.expneurol.2009.05.012_bib126","doi-asserted-by":"crossref","first-page":"2425","DOI":"10.1093\/hmg\/10.21.2425","article-title":"Centrosome disorganization in fibroblast cultures derived from R6\/2 Huntington's disease (HD) transgenic mice and HD patients","volume":"10","author":"Sathasivam","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib127","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/S0092-8674(00)81782-1","article-title":"Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions","volume":"95","author":"Saudou","year":"1998","journal-title":"Cell"},{"key":"10.1016\/j.expneurol.2009.05.012_bib128","doi-asserted-by":"crossref","first-page":"1194","DOI":"10.1038\/13518","article-title":"Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization","volume":"5","author":"Sawa","year":"1999","journal-title":"Nat. Med."},{"key":"10.1016\/j.expneurol.2009.05.012_bib129","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1007\/BF01851645","article-title":"Fatty acid patterns and glucose tolerance in Huntington's chorea","volume":"167","author":"Schubotz","year":"1976","journal-title":"Res. Exp. Med."},{"key":"10.1016\/j.expneurol.2009.05.012_bib130","first-page":"77","article-title":"Autonomic nervous system and sudden cardiac death. Experimental basis and clinical observations for post-myocardial infarction risk stratification","volume":"85","author":"Schwartz","year":"1992","journal-title":"Circulation"},{"key":"10.1016\/j.expneurol.2009.05.012_bib131","doi-asserted-by":"crossref","first-page":"1248","DOI":"10.1001\/archneur.56.10.1248","article-title":"Sympathetic skin response and heart rate variability in patients with Huntington disease","volume":"56","author":"Sharma","year":"1999","journal-title":"Arch. Neurol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib132","doi-asserted-by":"crossref","first-page":"14474","DOI":"10.1074\/jbc.M600364200","article-title":"Regulation of intracellular accumulation of mutant Huntingtin by Beclin 1","volume":"281","author":"Shibata","year":"2006","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.expneurol.2009.05.012_bib133","doi-asserted-by":"crossref","first-page":"1953","DOI":"10.1093\/hmg\/11.17.1953","article-title":"Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses","volume":"11","author":"Sipione","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib134","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1136\/jmg.29.12.911","article-title":"Causes of death in patients with Huntington's disease and in unaffected first degree relatives","volume":"29","author":"S\u00f8rensen","year":"1992","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib135","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1002\/1531-8249(200007)48:1<130::AID-ANA27>3.0.CO;2-8","article-title":"Weight loss in Huntington's disease","volume":"48","author":"Stoy","year":"2000","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib136","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":"10.1016\/j.expneurol.2009.05.012_bib137","doi-asserted-by":"crossref","first-page":"11758","DOI":"10.1523\/JNEUROSCI.2461-07.2007","article-title":"Expression profiling of Huntington's disease models suggests that brain-derived neurotrophic factor depletion plays a major role in striatal degeneration","volume":"27","author":"Strand","year":"2007","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2009.05.012_bib138","doi-asserted-by":"crossref","first-page":"e635","DOI":"10.1371\/journal.pone.0000635","article-title":"Polyglutamine expansion mutation yields a pathological epitope linked to nucleation of protein aggregate: determinant of Huntington's disease onset","volume":"2","author":"Sugaya","year":"2007","journal-title":"PLoS ONE"},{"key":"10.1016\/j.expneurol.2009.05.012_bib139","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":"10.1016\/j.expneurol.2009.05.012_bib140","doi-asserted-by":"crossref","first-page":"649","DOI":"10.1038\/sj.ejhg.5201562","article-title":"Huntington's disease: a transcriptional report card from the peripheral blood: can it measure disease progression in Huntington's disease?","volume":"14","author":"Tabchy","year":"2006","journal-title":"Eur. J. Hum. Genet."},{"key":"10.1016\/j.expneurol.2009.05.012_bib141","doi-asserted-by":"crossref","first-page":"1759","DOI":"10.1093\/brain\/awm044","article-title":"Microglial activation in presymptomatic Huntington's disease gene carriers","volume":"130","author":"Tai","year":"2007","journal-title":"Brain"},{"key":"10.1016\/j.expneurol.2009.05.012_bib142","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1046\/j.1471-4159.2002.00940.x","article-title":"Comparison of huntingtin proteolytic fragments in human lymphoblast cell lines and human brain","volume":"82","author":"Toneff","year":"2002","journal-title":"J. Neurochem."},{"key":"10.1016\/j.expneurol.2009.05.012_bib143","doi-asserted-by":"crossref","first-page":"1078","DOI":"10.1042\/BST20051078","article-title":"Signalling role of adipose tissue: adipokines and inflammation in obesity","volume":"33","author":"Trayhurn","year":"2005","journal-title":"Biochem. Soc. Trans."},{"key":"10.1016\/j.expneurol.2009.05.012_bib144","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1016\/j.nut.2003.10.007","article-title":"Assessment of the nutrition status of patients with Huntington's disease","volume":"20","author":"Trejo","year":"2004","journal-title":"Nutrition"},{"key":"10.1016\/j.expneurol.2009.05.012_bib145","doi-asserted-by":"crossref","first-page":"4252","DOI":"10.1111\/j.1742-4658.2008.06561.x","article-title":"Huntington's disease: revisiting the aggregation hypothesis in poly glutamine neurodegenerative diseases","volume":"275","author":"Truant","year":"2008","journal-title":"FEBS J."},{"key":"10.1016\/j.expneurol.2009.05.012_bib146","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":"10.1016\/j.expneurol.2009.05.012_bib147","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1016\/j.pneurobio.2006.09.005","article-title":"Cholesterol dysfunction in neurodegenerative diseases: is Huntington's disease in the list?","volume":"80","author":"Valenza","year":"2006","journal-title":"Prog. Neurobiol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib148","doi-asserted-by":"crossref","first-page":"9932","DOI":"10.1523\/JNEUROSCI.3355-05.2005","article-title":"Dysfunction of the cholesterol biosynthetic pathway in Huntington's disease","volume":"25","author":"Valenza","year":"2005","journal-title":"J. Neurosci."},{"key":"10.1016\/j.expneurol.2009.05.012_bib149","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.nbd.2007.07.029","article-title":"Increased metabolism in the R6\/2 mouse model of Huntington's disease","volume":"29","author":"Van der Burg","year":"2008","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.expneurol.2009.05.012_bib150","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1097\/00001756-200604240-00022","article-title":"Aggregate distribution in frontal and motor cortex in Huntington's disease brain","volume":"17","author":"Van Roon-Mom","year":"2006","journal-title":"NeuroReport"},{"key":"10.1016\/j.expneurol.2009.05.012_bib152","doi-asserted-by":"crossref","first-page":"2148","DOI":"10.1096\/fj.03-0079fje","article-title":"Aberrant A2A receptor function in peripheral blood cells in Huntington's disease","volume":"17","author":"Varani","year":"2003","journal-title":"FASEB J."},{"key":"10.1016\/j.expneurol.2009.05.012_bib153","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1016\/j.nbd.2007.03.011","article-title":"Biological abnormalities of peripheral A(2A) receptors in a large representation of polyglutamine disorders and Huntington's disease stages","volume":"27","author":"Varani","year":"2007","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.expneurol.2009.05.012_bib154","doi-asserted-by":"crossref","first-page":"1215","DOI":"10.1038\/nsmb860","article-title":"Hsp70 and Hsp40 attenuate formation of spherical and annular polyglutamine oligomers by partitioning monomer","volume":"11","author":"Wacker","year":"2004","journal-title":"Nat. Struct. Mol. Biol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib155","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":"10.1016\/j.expneurol.2009.05.012_bib156","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/0022-510X(80)90028-3","article-title":"Metabolic impairment and membrane abnormality in red cells from Huntington's disease","volume":"47","author":"Zanella","year":"1980","journal-title":"Neurol. Sci."},{"key":"10.1016\/j.expneurol.2009.05.012_bib157","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1016\/j.pneurobio.2007.01.003","article-title":"Role of brain-derived neurotrophic factor in Huntington's disease","volume":"81","author":"Zuccato","year":"2007","journal-title":"Prog. Neurobiol."},{"key":"10.1016\/j.expneurol.2009.05.012_bib158","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1038\/ng1219","article-title":"Huntingtin interacts with REST\/NRSF to modulate the transcription of NRSE-controlled neuronal genes","volume":"35","author":"Zuccato","year":"2003","journal-title":"Nat. Genet."}],"container-title":["Experimental Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0014488609001940?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0014488609001940?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2020,5,18]],"date-time":"2020-05-18T12:11:14Z","timestamp":1589803874000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0014488609001940"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,10]]},"references-count":157,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2009,10]]}},"alternative-id":["S0014488609001940"],"URL":"https:\/\/doi.org\/10.1016\/j.expneurol.2009.05.012","relation":{},"ISSN":["0014-4886"],"issn-type":[{"value":"0014-4886","type":"print"}],"subject":[],"published":{"date-parts":[[2009,10]]}}}