{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T03:51:30Z","timestamp":1792036290108,"version":"4.4.0"},"reference-count":51,"publisher":"Society for Neuroscience","issue":"11","license":[{"start":{"date-parts":[[2010,9,17]],"date-time":"2010-09-17T00:00:00Z","timestamp":1284681600000},"content-version":"vor","delay-in-days":184,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Neurosci."],"published-print":{"date-parts":[[2010,3,17]]},"abstract":"<jats:p>Huntington's disease (HD) is a neurodegenerative disorder caused by the expansion of a polyglutamine stretch in the protein huntingtin (Htt). HD neurons are dysfunctional at multiple levels and have increased susceptibility to stress and apoptotic stimuli. We have discovered that synthesis of the ganglioside GM1 is reduced in fibroblasts from HD patients and in cell and animal models of HD, and that decreased GM1 levels contribute to heighten HD cell susceptibility to apoptosis. The apoptotic susceptibility is recapitulated through inhibition of ganglioside synthesis in wild-type striatal cells, suggesting that decreased GM1 levels might be one of the key events leading to HD pathogenesis and progression. Administration of GM1 restores ganglioside levels in HD cells and promotes activation of AKT and phosphorylation of mutant Htt, leading to decreased mutant Htt toxicity and increased survival of HD cells. Our data identify GM1 as a potential treatment for HD.<\/jats:p>","DOI":"10.1523\/jneurosci.6348-09.2010","type":"journal-article","created":{"date-parts":[[2010,3,17]],"date-time":"2010-03-17T14:26:47Z","timestamp":1268836007000},"page":"4072-4080","source":"Crossref","is-referenced-by-count":141,"title":["Impaired Ganglioside Metabolism in Huntington's Disease and Neuroprotective Role of GM1"],"prefix":"10.1523","volume":"30","author":[{"given":"Vittorio","family":"Maglione","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Marchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alba","family":"Di Pardo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanne","family":"Lingrell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melanie","family":"Horkey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily","family":"Tidmarsh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simonetta","family":"Sipione","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"393","published-online":{"date-parts":[[2010,3,17]]},"reference":[{"key":"2023041303430800000_30.11.4072.1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2007.04713.x"},{"key":"2023041303430800000_30.11.4072.2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1998.tb09691.x"},{"key":"2023041303430800000_30.11.4072.3","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2005.06.005"},{"key":"2023041303430800000_30.11.4072.4","doi-asserted-by":"publisher","DOI":"10.1006\/exnr.2000.7504"},{"key":"2023041303430800000_30.11.4072.5","first-page":"CD004444","article-title":"Gangliosides for acute spinal cord injury","volume":"2","author":"Chinnock","year":"2005","journal-title":"Cochrane Database Syst Rev"},{"key":"2023041303430800000_30.11.4072.6","first-page":"215","article-title":"Brain white-matter volume loss and glucose hypometabolism precede the clinical symptoms of Huntington's disease","volume":"47","author":"Ciarmiello","year":"2006","journal-title":"J Nucl Med"},{"key":"2023041303430800000_30.11.4072.7","doi-asserted-by":"publisher","DOI":"10.1038\/35018098"},{"key":"2023041303430800000_30.11.4072.8","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9568.2005.03985.x"},{"key":"2023041303430800000_30.11.4072.9","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M607839200"},{"key":"2023041303430800000_30.11.4072.10","doi-asserted-by":"publisher","DOI":"10.1038\/nn1442"},{"key":"2023041303430800000_30.11.4072.11","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2007.05.003"},{"key":"2023041303430800000_30.11.4072.12","doi-asserted-by":"publisher","DOI":"10.1126\/science.1072613"},{"key":"2023041303430800000_30.11.4072.13","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.270.7.3074"},{"key":"2023041303430800000_30.11.4072.14","doi-asserted-by":"publisher","DOI":"10.1007\/s002210050573"},{"key":"2023041303430800000_30.11.4072.15","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.012540899"},{"key":"2023041303430800000_30.11.4072.16","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.72.7.2520"},{"key":"2023041303430800000_30.11.4072.17","doi-asserted-by":"publisher","DOI":"10.1016\/S1534-5807(02)00188-0"},{"key":"2023041303430800000_30.11.4072.18","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.030338"},{"key":"2023041303430800000_30.11.4072.19","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20071619"},{"key":"2023041303430800000_30.11.4072.20","doi-asserted-by":"publisher","DOI":"10.1016\/S0959-4388(00)00092-1"},{"key":"2023041303430800000_30.11.4072.21","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20041694"},{"key":"2023041303430800000_30.11.4072.22","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(85)90419-1"},{"key":"2023041303430800000_30.11.4072.23","doi-asserted-by":"publisher","DOI":"10.1194\/jlr.R800009-JLR200"},{"key":"2023041303430800000_30.11.4072.24","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200412071"},{"key":"2023041303430800000_30.11.4072.25","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81369-0"},{"key":"2023041303430800000_30.11.4072.26","doi-asserted-by":"publisher","DOI":"10.1007\/s00018-005-5188-y"},{"key":"2023041303430800000_30.11.4072.27","doi-asserted-by":"crossref","first-page":"825","DOI":"10.1161\/01.STR.21.5.825","article-title":"GM1 ganglioside therapy in acute ischemic stroke","volume":"21","author":"Oppenheimer","year":"1990","journal-title":"Stroke"},{"key":"2023041303430800000_30.11.4072.28","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1098-2396(200005)36:2<120::AID-SYN5>3.0.CO;2-Y"},{"key":"2023041303430800000_30.11.4072.29","doi-asserted-by":"crossref","first-page":"184","DOI":"10.1016\/j.bbamem.2008.09.001","article-title":"Glycosphingolipid behaviour in complex membranes","volume":"1788","author":"Prinetti","year":"2009","journal-title":"Biochim Biophys Acta"},{"key":"2023041303430800000_30.11.4072.30","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M511648200"},{"key":"2023041303430800000_30.11.4072.31","doi-asserted-by":"crossref","first-page":"3705","DOI":"10.1523\/JNEUROSCI.20-10-03705.2000","article-title":"Wild-type huntingtin protects from apoptosis upstream of caspase-3","volume":"20","author":"Rigamonti","year":"2000","journal-title":"J Neurosci"},{"key":"2023041303430800000_30.11.4072.32","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M513507200"},{"key":"2023041303430800000_30.11.4072.33","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1998.tb09688.x"},{"key":"2023041303430800000_30.11.4072.34","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.96.13.7532"},{"key":"2023041303430800000_30.11.4072.35","doi-asserted-by":"publisher","DOI":"10.1038\/ng1460"},{"key":"2023041303430800000_30.11.4072.36","doi-asserted-by":"publisher","DOI":"10.1006\/exnr.1998.6778"},{"key":"2023041303430800000_30.11.4072.37","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/11.17.1953"},{"key":"2023041303430800000_30.11.4072.38","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddg169"},{"key":"2023041303430800000_30.11.4072.39","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M110338200"},{"key":"2023041303430800000_30.11.4072.40","doi-asserted-by":"publisher","DOI":"10.1093\/glycob\/cwl052"},{"key":"2023041303430800000_30.11.4072.41","doi-asserted-by":"publisher","DOI":"10.1016\/j.mad.2005.09.012"},{"key":"2023041303430800000_30.11.4072.42","doi-asserted-by":"publisher","DOI":"10.1023\/B:GLYC.0000033627.02765.cc"},{"key":"2023041303430800000_30.11.4072.43","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(72)90661-0"},{"key":"2023041303430800000_30.11.4072.44","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/9.19.2799"},{"key":"2023041303430800000_30.11.4072.45","doi-asserted-by":"publisher","DOI":"10.1016\/j.brainres.2007.06.059"},{"key":"2023041303430800000_30.11.4072.46","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(07)60111-1"},{"key":"2023041303430800000_30.11.4072.47","doi-asserted-by":"publisher","DOI":"10.1016\/j.mcn.2008.09.007"},{"key":"2023041303430800000_30.11.4072.48","doi-asserted-by":"publisher","DOI":"10.1023\/B:GLYC.0000046273.68493.f7"},{"key":"2023041303430800000_30.11.4072.49","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.3635-05.2005"},{"key":"2023041303430800000_30.11.4072.50","doi-asserted-by":"publisher","DOI":"10.1194\/jlr.R300020-JLR200"},{"key":"2023041303430800000_30.11.4072.51","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddn281"}],"container-title":["The Journal of Neuroscience"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1523\/JNEUROSCI.6348-09.2010","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,13]],"date-time":"2023-04-13T15:51:11Z","timestamp":1681401071000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.jneurosci.org\/lookup\/doi\/10.1523\/JNEUROSCI.6348-09.2010"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,3,17]]},"references-count":51,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2010,3,17]]},"published-print":{"date-parts":[[2010,3,17]]}},"alternative-id":["10.1523\/JNEUROSCI.6348-09.2010"],"URL":"https:\/\/doi.org\/10.1523\/jneurosci.6348-09.2010","relation":{},"ISSN":["0270-6474","1529-2401"],"issn-type":[{"value":"0270-6474","type":"print"},{"value":"1529-2401","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,3,17]]}}}