{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T10:45:42Z","timestamp":1779187542137,"version":"3.51.4"},"reference-count":51,"publisher":"Public Library of Science (PLoS)","issue":"12","license":[{"start":{"date-parts":[[2011,12,7]],"date-time":"2011-12-07T00:00:00Z","timestamp":1323216000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0028409","type":"journal-article","created":{"date-parts":[[2011,12,7]],"date-time":"2011-12-07T22:08:40Z","timestamp":1323295720000},"page":"e28409","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":19,"title":["Onset and Progression of Behavioral and Molecular Phenotypes in a Novel Congenic R6\/2 Line Exhibiting Intergenerational CAG Repeat Stability"],"prefix":"10.1371","volume":"6","author":[{"given":"Randi-Michelle","family":"Cowin","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nghiem","family":"Bui","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Deanna","family":"Graham","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennie R.","family":"Green","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephan","family":"Grueninger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lisa A.","family":"Yuva-Paylor","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arsalan U.","family":"Syed","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Weiss","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard","family":"Paylor","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"340","published-online":{"date-parts":[[2011,12,7]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1038\/ng0893-398","article-title":"The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington&apos;s disease.","volume":"4","author":"SE Andrew","year":"1993","journal-title":"Nat Genet"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1038\/ng0893-387","article-title":"Trinucleotide repeat length instability and age of onset in Huntington&apos;s disease.","volume":"4","author":"M Duyao","year":"1993","journal-title":"Nat Genet"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1038\/ng0893-393","article-title":"Relationship between trinucleotide repeat expansion and phenotypic variation in Huntington&apos;s disease.","volume":"4","author":"RG Snell","year":"1993","journal-title":"Nat Genet"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"1547","DOI":"10.1093\/hmg\/2.10.1547","article-title":"Correlation between the onset age of Huntington&apos;s disease and length of the trinucleotide repeat in IT-15.","volume":"2","author":"OC Stine","year":"1993","journal-title":"Hum Mol Genet"},{"key":"ref5","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":"L Mangiarini","year":"1996","journal-title":"Cell"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1016\/j.nbd.2009.05.007","article-title":"Systematic behavioral evaluation of Huntington&apos;s disease transgenic and knock-in mouse models.","volume":"35","author":"L Menalled","year":"2009","journal-title":"Neurobiol Dis"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1002\/aur.168","article-title":"Modifying behavioral phenotypes in Fmr1KO mice: genetic background differences reveal autistic-like responses.","volume":"4","author":"CM Spencer","year":"2011","journal-title":"Autism Res"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/j.nbd.2006.12.010","article-title":"Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain.","volume":"26","author":"JM Van Raamsdonk","year":"2007","journal-title":"Neurobiol Dis"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1016\/j.nbd.2008.11.015","article-title":"Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6\/2 mice.","volume":"33","author":"AJ Morton","year":"2009","journal-title":"Neurobiol Dis"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1016\/j.nbd.2008.10.009","article-title":"CAG repeat lengths &gt; or = 335 attenuate the phenotype in the R6\/2 Huntington&apos;s disease transgenic mouse.","volume":"33","author":"I Dragatsis","year":"2009","journal-title":"Neurobiol Dis"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/S0896-6273(00)80764-3","article-title":"A YAC mouse model for Huntington&apos;s disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.","volume":"23","author":"JG Hodgson","year":"1999","journal-title":"Neuron"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"9112","DOI":"10.1523\/JNEUROSCI.21-23-09112.2001","article-title":"Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington&apos;s disease.","volume":"21","author":"GA Laforet","year":"2001","journal-title":"J Neurosci"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1002\/(SICI)1097-4547(19991115)58:4<515::AID-JNR5>3.0.CO;2-F","article-title":"Enhanced sensitivity to N-methyl-D-aspartate receptor activation in transgenic and knockin mouse models of Huntington&apos;s disease.","volume":"58","author":"MS Levine","year":"1999","journal-title":"J Neurosci Res"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1093\/hmg\/10.2.137","article-title":"Neurological abnormalities in a knock-in mouse model of Huntington&apos;s disease.","volume":"10","author":"CH Lin","year":"2001","journal-title":"Hum Mol Genet"},{"key":"ref15","first-page":"397","article-title":"Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin.","volume":"8","author":"G Schilling","year":"1999","journal-title":"Hum Mol Genet"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1093\/hmg\/8.5.763","article-title":"A Huntington&apos;s disease CAG expansion at the murine Hdh locus is unstable and associated with behavioural abnormalities in mice.","volume":"8","author":"PF Shelbourne","year":"1999","journal-title":"Hum Mol Genet"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1093\/hmg\/9.4.503","article-title":"Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice.","volume":"9","author":"VC Wheeler","year":"2000","journal-title":"Hum Mol Genet"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1016\/j.ab.2009.08.001","article-title":"Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington&apos;s disease.","volume":"395","author":"A Weiss","year":"2009","journal-title":"Anal Biochem"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1038\/ng0297-197","article-title":"Instability of highly expanded CAG repeats in mice transgenic for the Huntington&apos;s disease mutation.","volume":"15","author":"L Mangiarini","year":"1997","journal-title":"Nat Genet"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1007\/BF01067821","article-title":"Characterization of benzodiazepine-sensitive behaviors in the A\/J and C57BL\/6J inbred strains of mice.","volume":"24","author":"C Mathis","year":"1994","journal-title":"Behav Genet"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1016\/0091-3057(88)90413-3","article-title":"Thigmotaxis as a test for anxiolytic activity in rats.","volume":"31","author":"D Treit","year":"1988","journal-title":"Pharmacol Biochem Behav"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1006\/nlme.1995.1008","article-title":"Time-dependent deficits of rat&apos;s memory consolidation induced by tetrodotoxin injections into the caudate-putamen, nucleus accumbens, and globus pallidus.","volume":"63","author":"CA Lorenzini","year":"1995","journal-title":"Neurobiol Learn Mem"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1016\/0091-3057(85)90385-5","article-title":"Injections of atropine into the caudate nucleus impair the acquisition and the maintenance of passive avoidance.","volume":"22","author":"RA Prado-Alcala","year":"1985","journal-title":"Pharmacol Biochem Behav"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"2193","DOI":"10.1523\/JNEUROSCI.5473-08.2009","article-title":"Differential susceptibility to excitotoxic stress in YAC128 mouse models of Huntington disease between initiation and progression of disease.","volume":"29","author":"RK Graham","year":"2009","journal-title":"J Neurosci"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"2414","DOI":"10.1523\/JNEUROSCI.5687-08.2009","article-title":"Age-dependent alterations of corticostriatal activity in the YAC128 mouse model of Huntington disease.","volume":"29","author":"PR Joshi","year":"2009","journal-title":"J Neurosci"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/j.nbd.2008.09.014","article-title":"Intergenerational and striatal CAG repeat instability in Huntington&apos;s disease knock-in mice involve different DNA repair genes.","volume":"33","author":"E Dragileva","year":"2009","journal-title":"Neurobiol Dis"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1038\/86906","article-title":"Trinucleotide expansion in haploid germ cells by gap repair.","volume":"27","author":"IV Kovtun","year":"2001","journal-title":"Nat Genet"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"3057","DOI":"10.1093\/hmg\/ddh325","article-title":"Somatic deletion events occur during early embryonic development and modify the extent of CAG expansion in subsequent generations.","volume":"13","author":"IV Kovtun","year":"2004","journal-title":"Hum Mol Genet"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1038\/70598","article-title":"Msh2 deficiency prevents in vivo somatic instability of the CAG repeat in Huntington disease transgenic mice.","volume":"23","author":"K Manley","year":"1999","journal-title":"Nat Genet"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1038\/nsmb965","article-title":"(CAG)(n)-hairpin DNA binds to Msh2-Msh3 and changes properties of mismatch recognition.","volume":"12","author":"BA Owen","year":"2005","journal-title":"Nat Struct Mol Biol"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1128\/MCB.24.2.629-637.2004","article-title":"MSH2-dependent germinal CTG repeat expansions are produced continuously in spermatogonia from DM1 transgenic mice.","volume":"24","author":"C Savouret","year":"2004","journal-title":"Mol Cell Biol"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"e1000482","DOI":"10.1371\/journal.pgen.1000482","article-title":"MSH2 ATPase domain mutation affects CTG*CAG repeat instability in transgenic mice.","volume":"5","author":"S Tome","year":"2009","journal-title":"PLoS Genet"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1093\/hmg\/11.2.191","article-title":"Somatic expansion behaviour of the (CTG)n repeat in myotonic dystrophy knock-in mice is differentially affected by Msh3 and Msh6 mismatch-repair proteins.","volume":"11","author":"WJ van den Broek","year":"2002","journal-title":"Hum Mol Genet"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1093\/hmg\/ddg056","article-title":"Mismatch repair gene Msh2 modifies the timing of early disease in Hdh(Q111) striatum.","volume":"12","author":"VC Wheeler","year":"2003","journal-title":"Hum Mol Genet"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.nbd.2005.01.024","article-title":"Early behavioral deficits in R6\/2 mice suitable for use in preclinical drug testing.","volume":"20","author":"MA Hickey","year":"2005","journal-title":"Neurobiol Dis"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"10428","DOI":"10.1523\/JNEUROSCI.19-23-10428.1999","article-title":"Selective discrimination learning impairments in mice expressing the human Huntington&apos;s disease mutation.","volume":"19","author":"LA Lione","year":"1999","journal-title":"J Neurosci"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1136\/jnnp.58.2.192","article-title":"Impaired prepulse inhibition of acoustic and tactile startle response in patients with Huntington&apos;s disease.","volume":"58","author":"NR Swerdlow","year":"1995","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"ref38","article-title":"Longitudinal analysis of the behavioural phenotype in YAC128 (C57BL\/6J) Huntington&apos;s disease transgenic mice.","author":"S Brooks","year":"2010","journal-title":"Brain Res Bull"},{"key":"ref39","article-title":"Longitudinal analysis of the behavioural phenotype in Hdh((CAG)150) Huntington&apos;s disease knock-in mice.","author":"S Brooks","year":"2010","journal-title":"Brain Res Bull"},{"key":"ref40","article-title":"Longitudinal analysis of the behavioural phenotype in R6\/1 (C57BL\/6J) Huntington&apos;s disease transgenic mice.","author":"SP Brooks","year":"2011","journal-title":"Brain Res Bull"},{"key":"ref41","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&apos;s disease mutation.","volume":"19","author":"RJ Carter","year":"1999","journal-title":"J Neurosci"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"4169","DOI":"10.1523\/JNEUROSCI.0590-05.2005","article-title":"Cognitive dysfunction precedes neuropathology and motor abnormalities in the YAC128 mouse model of Huntington&apos;s disease.","volume":"25","author":"JM Van Raamsdonk","year":"2005","journal-title":"J Neurosci"},{"key":"ref43","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1016\/j.nlm.2007.10.009","article-title":"Rigidity in social and emotional memory in the R6\/2 mouse model of Huntington&apos;s disease.","volume":"89","author":"A Ciamei","year":"2008","journal-title":"Neurobiol Learn Mem"},{"key":"ref44","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1016\/j.nbd.2011.02.005","article-title":"\u201cBrain training\u201d improves cognitive performance and survival in a transgenic mouse model of Huntington&apos;s disease.","volume":"42","author":"NI Wood","year":"2011","journal-title":"Neurobiol Dis"},{"key":"ref45","doi-asserted-by":"crossref","first-page":"1571","DOI":"10.1016\/j.neuroscience.2007.03.036","article-title":"Dopamine-dependent long term potentiation in the dorsal striatum is reduced in the R6\/2 mouse model of Huntington&apos;s disease.","volume":"146","author":"VW Kung","year":"2007","journal-title":"Neuroscience"},{"key":"ref46","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/j.bbr.2006.01.003","article-title":"Behaviour changes in a transgenic model of Huntington&apos;s disease.","volume":"169","author":"P Klivenyi","year":"2006","journal-title":"Behav Brain Res"},{"key":"ref47","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1038\/ng1197-280","article-title":"Theoretical and empirical issues for marker-assisted breeding of congenic mouse strains.","volume":"17","author":"P Markel","year":"1997","journal-title":"Nat Genet"},{"key":"ref48","doi-asserted-by":"crossref","first-page":"472","DOI":"10.1016\/S0167-5699(97)01126-2","article-title":"Speed congenics: a classic technique in the fast lane (relatively speaking).","volume":"18","author":"E Wakeland","year":"1997","journal-title":"Immunol Today"},{"key":"ref49","doi-asserted-by":"crossref","first-page":"1816","DOI":"10.1093\/nar\/29.9.e45","article-title":"A new mathematical model for relative quantification in real-time RT-PCR.","volume":"29","author":"MW Pfaffl","year":"2001","journal-title":"Nucleic Acids Res"},{"key":"ref50","doi-asserted-by":"crossref","first-page":"RESEARCH0034.1","DOI":"10.1186\/gb-2002-3-7-research0034","article-title":"Accurate normalization of real-time quantitative RT-PCR data by geometric averaging of multiple internal control genes.","volume":"3","author":"J Vandesompele","year":"2002","journal-title":"Genome Biol"},{"key":"ref51","doi-asserted-by":"crossref","first-page":"1869","DOI":"10.1093\/nar\/30.9.e36","article-title":"Relative expression software tool (REST) for group-wise comparison and statistical analysis of relative expression results in real-time PCR.","volume":"30","author":"MW Pfaffl","year":"2002","journal-title":"Nucleic Acids Res"}],"container-title":["PLoS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0028409","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,5,9]],"date-time":"2020-05-09T01:44:40Z","timestamp":1588988680000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0028409"}},"subtitle":[],"editor":[{"given":"Ya-Ping","family":"Tang","sequence":"first","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2011,12,7]]},"references-count":51,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2011,12,7]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0028409","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,12,7]]}}}