{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T19:17:00Z","timestamp":1791919020638,"version":"4.3.4"},"reference-count":45,"publisher":"Oxford University Press (OUP)","issue":"11","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2014,6,1]]},"DOI":"10.1093\/hmg\/ddu010","type":"journal-article","created":{"date-parts":[[2014,1,17]],"date-time":"2014-01-17T01:28:33Z","timestamp":1389922113000},"page":"2995-3007","source":"Crossref","is-referenced-by-count":140,"title":["A potent and selective Sirtuin 1 inhibitor alleviates pathology in multiple animal and cell models of Huntington's disease"],"prefix":"10.1093","volume":"23","author":[{"given":"Marianne R.","family":"Smith","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adeela","family":"Syed","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tamas","family":"Lukacsovich","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judy","family":"Purcell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brett A.","family":"Barbaro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shane A.","family":"Worthge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen R.","family":"Wei","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Pollio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Letizia","family":"Magnoni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carla","family":"Scali","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luisa","family":"Massai","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Franceschini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michela","family":"Camarri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Gianfriddo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Enrica","family":"Diodato","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Russell","family":"Thomas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ozgun","family":"Gokce","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.J.","family":"Tabrizi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Caricasole","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernard","family":"Landwehrmeyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liliana","family":"Menalled","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carol","family":"Murphy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sylvie","family":"Ramboz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruth","family":"Luthi-Carter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Goran","family":"Westerberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. Lawrence","family":"Marsh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2014,1,16]]},"reference":[{"key":"key\n\t\t\t\t20170522141655_DDU010C1","doi-asserted-by":"crossref","first-page":"10720","DOI":"10.1523\/JNEUROSCI.2126-08.2008","article-title":"Huntingtin modulates transcription, occupies gene promoters in vivo, and binds directly to DNA in a polyglutamine-dependent manner","volume":"28","author":"Benn","year":"2008","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522141655_DDU010C2","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\t20170522141655_DDU010C3","doi-asserted-by":"crossref","first-page":"e41423","DOI":"10.1371\/journal.pone.0041423","article-title":"Genome-wide histone acetylation is altered in a transgenic mouse model of Huntington's disease","volume":"7","author":"McFarland","year":"2012","journal-title":"PLoS ONE"},{"key":"key\n\t\t\t\t20170522141655_DDU010C4","doi-asserted-by":"crossref","first-page":"9418","DOI":"10.1523\/JNEUROSCI.23-28-09418.2003","article-title":"Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice","volume":"23","author":"Ferrante","year":"2003","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522141655_DDU010C5","doi-asserted-by":"crossref","first-page":"330","DOI":"10.1038\/ncpneuro0199","article-title":"Mechanisms of disease: Histone modifications in Huntington's disease","volume":"2","author":"Sadri-Vakili","year":"2006","journal-title":"Nat. Clin. Pract. Neurol."},{"key":"key\n\t\t\t\t20170522141655_DDU010C6","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1038\/35099568","article-title":"Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila","volume":"413","author":"Steffan","year":"2001","journal-title":"Nature"},{"key":"key\n\t\t\t\t20170522141655_DDU010C7","doi-asserted-by":"crossref","first-page":"1139","DOI":"10.1016\/j.bcp.2004.05.034","article-title":"Histone deacetylase inhibitors open new doors in cancer therapy","volume":"68","author":"McLaughlin","year":"2004","journal-title":"Biochem. Pharmacol."},{"key":"key\n\t\t\t\t20170522141655_DDU010C8","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1080\/07853890701408194","article-title":"Sirtuins: the \u2018magnificent seven\u2019, function, metabolism and longevity","volume":"39","author":"Dali-Youcef","year":"2007","journal-title":"Ann. Med."},{"key":"key\n\t\t\t\t20170522141655_DDU010C9","doi-asserted-by":"crossref","first-page":"3767","DOI":"10.1093\/hmg\/ddn273","article-title":"Inhibition of specific HDACs and sirtuins suppresses pathogenesis in a Drosophila model of Huntington's disease","volume":"17","author":"Pallos","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522141655_DDU010C10","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1016\/j.cmet.2008.05.004","article-title":"SirT1 inhibition reduces IGF-I\/IRS-2\/Ras\/ERK1\/2 signaling and protects neurons","volume":"8","author":"Li","year":"2008","journal-title":"Cell Metab."},{"key":"key\n\t\t\t\t20170522141655_DDU010C11","doi-asserted-by":"crossref","first-page":"2011","DOI":"10.1093\/hmg\/ddh214","article-title":"Comparison of pathways controlling toxicity in the eye and brain in Drosophila models of human neurodegenerative diseases","volume":"13","author":"Ghosh","year":"2004","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522141655_DDU010C12","doi-asserted-by":"crossref","first-page":"11500","DOI":"10.1523\/JNEUROSCI.3203-08.2008","article-title":"Nicotinamide restores cognition in Alzheimer's disease transgenic mice via a mechanism involving sirtuin inhibition and selective reduction of Thr231-phosphotau","volume":"28","author":"Green","year":"2008","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522141655_DDU010C13","doi-asserted-by":"crossref","first-page":"4607","DOI":"10.1111\/j.1742-4658.2005.04862.x","article-title":"Small molecule regulation of Sir2 protein deacetylases","volume":"272","author":"Grubisha","year":"2005","journal-title":"FEBS J."},{"key":"key\n\t\t\t\t20170522141655_DDU010C14","doi-asserted-by":"crossref","first-page":"8045","DOI":"10.1021\/jm050522v","article-title":"Discovery of indoles as potent and selective inhibitors of the deacetylase SIRT1","volume":"48","author":"Napper","year":"2005","journal-title":"J. Med. Chem."},{"key":"key\n\t\t\t\t20170522141655_DDU010C15","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1016\/j.molcel.2004.12.032","article-title":"Chemical activation of Sir2-dependent silencing by relief of nicotinamide inhibition","volume":"17","author":"Sauve","year":"2005","journal-title":"Mol. Cell"},{"key":"key\n\t\t\t\t20170522141655_DDU010C16","doi-asserted-by":"crossref","first-page":"38837","DOI":"10.1074\/jbc.M106779200","article-title":"Identification of a class of small molecule inhibitors of the sirtuin family of NAD-dependent deacetylases by phenotypic screening","volume":"276","author":"Grozinger","year":"2001","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522141655_DDU010C17","doi-asserted-by":"crossref","first-page":"7789","DOI":"10.1021\/jm050100l","article-title":"Design, synthesis, and biological evaluation of sirtinol analogues as class III histone\/protein deacetylase (Sirtuin) inhibitors","volume":"48","author":"Mai","year":"2005","journal-title":"J. Med. Chem."},{"key":"key\n\t\t\t\t20170522141655_DDU010C18","doi-asserted-by":"crossref","first-page":"5219","DOI":"10.1002\/anie.200605207","article-title":"Phloroglucinol derivatives guttiferone G, aristoforin, and hyperforin: inhibitors of human sirtuins SIRT1 and SIRT2","volume":"46","author":"Gey","year":"2007","journal-title":"Angew. Chem."},{"key":"key\n\t\t\t\t20170522141655_DDU010C19","doi-asserted-by":"crossref","first-page":"963","DOI":"10.1021\/jm301431y","article-title":"The 2.5 A crystal structure of the SIRT1 catalytic domain bound to nicotinamide adenine dinucleotide (NAD+) and an indole (EX527 analogue) reveals a novel mechanism of histone deacetylase inhibition","volume":"56","author":"Zhao","year":"2013","journal-title":"J. Med. Chem."},{"key":"key\n\t\t\t\t20170522141655_DDU010C20","doi-asserted-by":"crossref","first-page":"E2772","DOI":"10.1073\/pnas.1303628110","article-title":"Ex-527 inhibits Sirtuins by exploiting their unique NAD+-dependent deacetylation mechanism","volume":"110","author":"Gertz","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522141655_DDU010C21","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1007\/978-1-62703-438-8_3","article-title":"Morphometric analysis of Huntington's disease neurodegeneration in Drosophila","volume":"1017","author":"Song","year":"2013","journal-title":"Methods Mol. Biol."},{"key":"key\n\t\t\t\t20170522141655_DDU010C22","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1016\/j.neuron.2006.09.025","article-title":"Drosophila in the study of neurodegenerative disease","volume":"52","author":"Marsh","year":"2006","journal-title":"Neuron"},{"key":"key\n\t\t\t\t20170522141655_DDU010C23","doi-asserted-by":"crossref","first-page":"4251","DOI":"10.1242\/jcs.073783","article-title":"SIRT2 regulates NF-kappaB dependent gene expression through deacetylation of p65 Lys310","volume":"123","author":"Rothgiesser","year":"2010","journal-title":"J. Cell Sci."},{"key":"key\n\t\t\t\t20170522141655_DDU010C24","doi-asserted-by":"crossref","first-page":"1829","DOI":"10.1093\/hmg\/10.17.1829","article-title":"Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease","volume":"10","author":"Wyttenbach","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522141655_DDU010C25","doi-asserted-by":"crossref","first-page":"903","DOI":"10.1038\/mt.sj.6300141","article-title":"Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease","volume":"15","author":"Perrin","year":"2007","journal-title":"Mol. Ther."},{"key":"key\n\t\t\t\t20170522141655_DDU010C26","doi-asserted-by":"crossref","first-page":"9723","DOI":"10.1523\/JNEUROSCI.3044-08.2008","article-title":"Dysregulation of gene expression in primary neuron models of Huntington's disease shows that polyglutamine-related effects on the striatal transcriptome may not be dependent on brain circuitry","volume":"28","author":"Runne","year":"2008","journal-title":"J. Neurosci."},{"key":"key\n\t\t\t\t20170522141655_DDU010C27","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1016\/j.nbd.2005.05.017","article-title":"Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment","volume":"20","author":"Zala","year":"2005","journal-title":"Neurobiol. Dis."},{"key":"key\n\t\t\t\t20170522141655_DDU010C28","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1016\/S0092-8674(00)81369-0","article-title":"Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice","volume":"87","author":"Mangiarini","year":"1996","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522141655_DDU010C29","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1016\/j.nbd.2009.05.007","article-title":"Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models","volume":"35","author":"Menalled","year":"2009","journal-title":"Neurobiol. Dis."},{"key":"key\n\t\t\t\t20170522141655_DDU010C30","doi-asserted-by":"crossref","first-page":"e9793","DOI":"10.1371\/journal.pone.0009793","article-title":"Comprehensive behavioral testing in the R6\/2 mouse model of Huntington's disease shows no benefit from CoQ10 or minocycline","volume":"5","author":"Menalled","year":"2010","journal-title":"PLoS ONE"},{"key":"key\n\t\t\t\t20170522141655_DDU010C31","doi-asserted-by":"crossref","first-page":"2366","DOI":"10.1073\/pnas.1221891110","article-title":"Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease","volume":"110","author":"Sathasivam","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522141655_DDU010C32","doi-asserted-by":"crossref","first-page":"1666","DOI":"10.1016\/j.bbapap.2009.10.022","article-title":"SIRT1-dependent regulation of chromatin and transcription: linking NAD(+) metabolism and signaling to the control of cellular functions","volume":"1804","author":"Zhang","year":"2010","journal-title":"Biochim. Biophys. Acta"},{"key":"key\n\t\t\t\t20170522141655_DDU010C33","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/j.molcel.2004.08.031","article-title":"Human SirT1 interacts with histone H1 and promotes formation of facultative heterochromatin","volume":"16","author":"Vaquero","year":"2004","journal-title":"Mol. Cell"},{"key":"key\n\t\t\t\t20170522141655_DDU010C34","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1016\/S0092-8674(01)00527-X","article-title":"hSIR2(SIRT1) functions as an NAD-dependent p53 deacetylase","volume":"107","author":"Vaziri","year":"2001","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522141655_DDU010C35","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1016\/S0092-8674(04)00126-6","article-title":"Mammalian SIRT1 represses forkhead transcription factors","volume":"116","author":"Motta","year":"2004","journal-title":"Cell"},{"key":"key\n\t\t\t\t20170522141655_DDU010C36","doi-asserted-by":"crossref","first-page":"1021","DOI":"10.1038\/sj.emboj.7600570","article-title":"Suppression of FOXO1 activity by FHL2 through SIRT1-mediated deacetylation","volume":"24","author":"Yang","year":"2005","journal-title":"EMBO J."},{"key":"key\n\t\t\t\t20170522141655_DDU010C37","doi-asserted-by":"crossref","first-page":"2369","DOI":"10.1038\/sj.emboj.7600244","article-title":"Modulation of NF-kappaB-dependent transcription and cell survival by the SIRT1 deacetylase","volume":"23","author":"Yeung","year":"2004","journal-title":"EMBO J."},{"key":"key\n\t\t\t\t20170522141655_DDU010C38","doi-asserted-by":"crossref","first-page":"16456","DOI":"10.1074\/jbc.M501485200","article-title":"SIRT1 functionally interacts with the metabolic regulator and transcriptional coactivator PGC-1{alpha}","volume":"280","author":"Nemoto","year":"2005","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522141655_DDU010C39","doi-asserted-by":"crossref","first-page":"10264","DOI":"10.1074\/jbc.M408748200","article-title":"SIRT1 deacetylation and repression of p300 involves lysine residues 1020\/1024 within the cell cycle regulatory domain 1","volume":"280","author":"Bouras","year":"2005","journal-title":"J. Biol. Chem."},{"key":"key\n\t\t\t\t20170522141655_DDU010C40","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1093\/hmg\/ddm315","article-title":"Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases","volume":"17","author":"Branco","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"key\n\t\t\t\t20170522141655_DDU010C41","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1038\/nm.2559","article-title":"Sirt1 mediates neuroprotection from mutant huntingtin by activation of the TORC1 and CREB transcriptional pathway","volume":"18","author":"Jeong","year":"2012","journal-title":"Nat. Med."},{"key":"key\n\t\t\t\t20170522141655_DDU010C42","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1038\/nm.2558","article-title":"Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets","volume":"18","author":"Jiang","year":"2012","journal-title":"Nat. Med."},{"key":"key\n\t\t\t\t20170522141655_DDU010C43","doi-asserted-by":"crossref","first-page":"784","DOI":"10.1016\/j.bbrc.2009.07.119","article-title":"Sirtuin 1 overexpression mice show a reference memory deficit, but not neuroprotection","volume":"387","author":"Kakefuda","year":"2009","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"key\n\t\t\t\t20170522141655_DDU010C44","doi-asserted-by":"crossref","first-page":"8673","DOI":"10.1073\/pnas.0803837105","article-title":"Sir2 mediates apoptosis through JNK-dependent pathways in Drosophila","volume":"105","author":"Griswold","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"key\n\t\t\t\t20170522141655_DDU010C45","doi-asserted-by":"crossref","first-page":"11458","DOI":"10.1074\/jbc.M109.087585","article-title":"SIRT1 regulates autoacetylation and histone acetyltransferase activity of TIP60","volume":"285","author":"Wang","year":"2010","journal-title":"J. Biol. Chem."}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/hmg\/article-pdf\/23\/11\/2995\/13917995\/ddu010.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,8,6]],"date-time":"2019-08-06T18:34:28Z","timestamp":1565116468000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article-lookup\/doi\/10.1093\/hmg\/ddu010"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,1,16]]},"references-count":45,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2014,1,16]]},"published-print":{"date-parts":[[2014,6,1]]}},"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddu010","relation":{},"ISSN":["1460-2083","0964-6906"],"issn-type":[{"value":"1460-2083","type":"electronic"},{"value":"0964-6906","type":"print"}],"subject":[],"published":{"date-parts":[[2014,1,16]]}}}