{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T15:14:45Z","timestamp":1792250085396,"version":"4.4.0"},"reference-count":28,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2008,11,1]],"date-time":"2008-11-01T00:00:00Z","timestamp":1225497600000},"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":["Behavioural Brain Research"],"published-print":{"date-parts":[[2008,11]]},"DOI":"10.1016\/j.bbr.2008.06.016","type":"journal-article","created":{"date-parts":[[2008,6,21]],"date-time":"2008-06-21T04:21:20Z","timestamp":1214022080000},"page":"315-319","source":"Crossref","is-referenced-by-count":28,"title":["Early deficits in motor coordination and cognitive dysfunction in a mouse model of the neurodegenerative lysosomal storage disorder, Sandhoff disease"],"prefix":"10.1016","volume":"193","author":[{"given":"Maria","family":"Gulinello","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fengying","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kostantin","family":"Dobrenis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.bbr.2008.06.016_bib1","doi-asserted-by":"crossref","first-page":"506","DOI":"10.1016\/j.nbd.2004.04.012","article-title":"Improved outcome of N-butyldeoxygalactonojirimycin-mediated substrate reduction therapy in a mouse model of Sandhoff disease","volume":"16","author":"Andersson","year":"2004","journal-title":"Neurobiol Dis"},{"key":"10.1016\/j.bbr.2008.06.016_bib2","doi-asserted-by":"crossref","first-page":"1841","DOI":"10.1038\/sj.gt.3302081","article-title":"Widespread distribution of beta-hexosaminidase activity in the brain of a Sandhoff mouse model after coinjection of adenoviral vector and mannitol","volume":"10","author":"Bourgoin","year":"2003","journal-title":"Gene Ther"},{"key":"10.1016\/j.bbr.2008.06.016_bib3","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/j.nlm.2004.01.001","article-title":"Short and long-term motor skill learning in an accelerated rotarod training paradigm","volume":"81","author":"Buitrago","year":"2004","journal-title":"Neurobiol Learn Mem"},{"key":"10.1016\/j.bbr.2008.06.016_bib4","doi-asserted-by":"crossref","first-page":"10373","DOI":"10.1073\/pnas.0603765103","article-title":"Effective gene therapy in an authentic model of Tay-Sachs-related diseases","volume":"103","author":"Cachon-Gonzalez","year":"2006","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/j.bbr.2008.06.016_bib5","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1016\/S0149-7634(01)00011-2","article-title":"A detailed ethological analysis of the mouse open field test: effects of diazepam, chlordiazepoxide and an extremely low frequency pulsed magnetic field","volume":"25","author":"Choleris","year":"2001","journal-title":"Neurosci Biobehav Rev"},{"key":"10.1016\/j.bbr.2008.06.016_bib6","doi-asserted-by":"crossref","first-page":"8853","DOI":"10.1523\/JNEUROSCI.20-23-08853.2000","article-title":"Impaired recognition memory in rats after damage to the hippocampus","volume":"20","author":"Clark","year":"2000","journal-title":"J Neurosci"},{"key":"10.1016\/j.bbr.2008.06.016_bib7","doi-asserted-by":"crossref","first-page":"1294","DOI":"10.1111\/j.1471-4159.2007.04525.x","article-title":"Neurochemical, morphological, and neurophysiological abnormalities in retina of Sandhoff and GM1 gangliosidosis mice","volume":"101","author":"Denny","year":"2007","journal-title":"J Neurochem"},{"key":"10.1016\/j.bbr.2008.06.016_bib8","doi-asserted-by":"crossref","first-page":"1028","DOI":"10.1002\/jnr.20798","article-title":"Influence of caloric restriction on motor behavior, longevity, and brain lipid composition in Sandhoff disease mice","volume":"83","author":"Denny","year":"2006","journal-title":"J Neurosci Res"},{"key":"10.1016\/j.bbr.2008.06.016_bib9","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/S0166-4328(05)80315-8","article-title":"A new one-trial test for neurobiological studies of memory in rats. III. Spatial vs. non-spatial working memory","volume":"51","author":"Ennaceur","year":"1992","journal-title":"Behav Brain Res"},{"key":"10.1016\/j.bbr.2008.06.016_bib10","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1001\/archneur.62.6.989","article-title":"The natural history of cognitive dysfunction in late-onset GM2 gangliosidosis","volume":"62","author":"Frey","year":"2005","journal-title":"Arch Neurol"},{"key":"10.1016\/j.bbr.2008.06.016_bib11","series-title":"The metabolic and molecular bases of inherited disease","first-page":"3827","article-title":"The GM2 gangliosidoses","author":"Gravel","year":"2001"},{"key":"10.1016\/j.bbr.2008.06.016_bib12","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1007\/BF00401402","article-title":"Comprehensive observational assessment. Ia. A systematic, quantitative procedure for assessing the behavioral and physiologic state of the mouse","volume":"13","author":"Irwin","year":"1968","journal-title":"Psychopharmacologia"},{"key":"10.1016\/j.bbr.2008.06.016_bib13","doi-asserted-by":"crossref","first-page":"6388","DOI":"10.1073\/pnas.96.11.6388","article-title":"Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin","volume":"96","author":"Jeyakumar","year":"1999","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/j.bbr.2008.06.016_bib14","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1182\/blood.V97.1.327","article-title":"Enhanced survival in Sandhoff disease mice receiving a combination of substrate deprivation therapy and bone marrow transplantation","volume":"97","author":"Jeyakumar","year":"2001","journal-title":"Blood"},{"key":"10.1016\/j.bbr.2008.06.016_bib15","doi-asserted-by":"crossref","first-page":"642","DOI":"10.1002\/ana.20242","article-title":"NSAIDS increase survival in the Sandhoff disease mouse: synergy with N-butyldeoxynojirimycin","volume":"56","author":"Jeyakumar","year":"2004","journal-title":"Ann Neurol"},{"key":"10.1016\/j.bbr.2008.06.016_bib16","doi-asserted-by":"crossref","first-page":"974","DOI":"10.1093\/brain\/awg089","article-title":"Central nervous system inflammation is a hallmark of pathogenesis in mouse models of GM1 and GM2 gangliosidosis","volume":"126","author":"Jeyakumar","year":"2003","journal-title":"Brain"},{"key":"10.1016\/j.bbr.2008.06.016_bib17","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1037\/0894-4105.10.1.74","article-title":"Greater relative impairment of object recognition than of visuospatial abilities in Alzheimer's disease","volume":"10","author":"Kurylo","year":"1996","journal-title":"Neuropsychology"},{"key":"10.1016\/j.bbr.2008.06.016_bib18","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1016\/j.molbrainres.2004.10.026","article-title":"Beta-hexosaminidase lentiviral vectors: transfer into the CNS via systemic administration","volume":"133","author":"Kyrkanides","year":"2005","journal-title":"Brain Res Mol Brain Res"},{"key":"10.1016\/j.bbr.2008.06.016_bib19","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1023\/A:1021003600900","article-title":"Motor coordination, exploration, and spatial learning in a natural mouse mutation (nervous) with Purkinje cell degeneration","volume":"33","author":"Lalonde","year":"2003","journal-title":"Behav Genet"},{"key":"10.1016\/j.bbr.2008.06.016_bib20","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1038\/nm1548","article-title":"Stem cells act through multiple mechanisms to benefit mice with neurodegenerative metabolic disease","volume":"13","author":"Lee","year":"2007","journal-title":"Nat Med"},{"key":"10.1016\/j.bbr.2008.06.016_bib21","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1176\/jnp.10.1.10","article-title":"Neuropsychiatric aspects of the adult variant of Tay-Sachs disease","volume":"10","author":"MacQueen","year":"1998","journal-title":"J Neuropsychiatry Clin Neurosci"},{"key":"10.1016\/j.bbr.2008.06.016_bib22","doi-asserted-by":"crossref","first-page":"1881","DOI":"10.1172\/JCI2127","article-title":"Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice","volume":"101","author":"Norflus","year":"1998","journal-title":"J Clin Invest"},{"key":"10.1016\/j.bbr.2008.06.016_bib23","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1093\/hmg\/5.1.1","article-title":"Dramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseases","volume":"5","author":"Phaneuf","year":"1996","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.bbr.2008.06.016_bib24","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1016\/S0304-3940(01)01885-7","article-title":"SHIRPA, a protocol for behavioral assessment: validation for longitudinal study of neurological dysfunction in mice","volume":"306","author":"Rogers","year":"2001","journal-title":"Neurosci Lett"},{"key":"10.1016\/j.bbr.2008.06.016_bib25","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1038\/ng1095-170","article-title":"Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism","volume":"11","author":"Sango","year":"1995","journal-title":"Nat Genet"},{"key":"10.1016\/j.bbr.2008.06.016_bib26","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1177\/0269881105051524","article-title":"The mouse beam walking assay offers improved sensitivity over the mouse rotarod in determining motor coordination deficits induced by benzodiazepines","volume":"19","author":"Stanley","year":"2005","journal-title":"J Psychopharmacol"},{"key":"10.1016\/j.bbr.2008.06.016_bib27","doi-asserted-by":"crossref","first-page":"8425","DOI":"10.1073\/pnas.0400625101","article-title":"Deletion of macrophage-inflammatory protein 1alpha retards neurodegeneration in Sandhoff disease mice","volume":"101","author":"Wu","year":"2004","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/j.bbr.2008.06.016_bib28","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1172\/JCI200419639","article-title":"Possible role of autoantibodies in the pathophysiology of GM2 gangliosidoses","volume":"113","author":"Yamaguchi","year":"2004","journal-title":"J Clin Invest"}],"container-title":["Behavioural Brain Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0166432808003112?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0166432808003112?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2019,5,12]],"date-time":"2019-05-12T03:44:56Z","timestamp":1557632696000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0166432808003112"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,11]]},"references-count":28,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2008,11]]}},"alternative-id":["S0166432808003112"],"URL":"https:\/\/doi.org\/10.1016\/j.bbr.2008.06.016","relation":{},"ISSN":["0166-4328"],"issn-type":[{"value":"0166-4328","type":"print"}],"subject":[],"published":{"date-parts":[[2008,11]]}}}