{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T04:54:00Z","timestamp":1790830440886,"version":"4.1.0"},"reference-count":72,"publisher":"Oxford University Press (OUP)","issue":"17","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hum. Mol. Genet."],"published-print":{"date-parts":[[2016,9,1]]},"DOI":"10.1093\/hmg\/ddw212","type":"journal-article","created":{"date-parts":[[2016,7,5]],"date-time":"2016-07-05T00:58:51Z","timestamp":1467680331000},"page":"3654-3675","source":"Crossref","is-referenced-by-count":134,"title":["An enhanced Q175 knock-in mouse model of Huntington disease with higher mutant huntingtin levels and accelerated disease phenotypes"],"prefix":"10.1093","volume":"25","author":[{"given":"Amber L.","family":"Southwell","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy","family":"Smith-Dijak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chris","family":"Kay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marja","family":"Sepers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erika B.","family":"Villanueva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew P.","family":"Parsons","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuanyun","family":"Xie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Anderson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Boguslaw","family":"Felczak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine","family":"Waltl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seunghyun","family":"Ko","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daphne","family":"Cheung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louisa","family":"Dal Cengio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ramy","family":"Slama","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eugenia","family":"Petoukhov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynn A.","family":"Raymond","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael R.","family":"Hayden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2016,7,4]]},"reference":[{"key":"2017010407302572000_25.17.3654.1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2012-304636"},{"key":"2017010407302572000_25.17.3654.2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.25717"},{"key":"2017010407302572000_25.17.3654.3","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(10)70245-3"},{"key":"2017010407302572000_25.17.3654.4","doi-asserted-by":"publisher","DOI":"10.1002\/mds.26331"},{"key":"2017010407302572000_25.17.3654.5","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(93)90585-E"},{"key":"2017010407302572000_25.17.3654.6","doi-asserted-by":"publisher","DOI":"10.1038\/ng0893-398"},{"key":"2017010407302572000_25.17.3654.7","doi-asserted-by":"publisher","DOI":"10.1038\/ng1297-404"},{"key":"2017010407302572000_25.17.3654.8","doi-asserted-by":"publisher","DOI":"10.1002\/cne.10776"},{"key":"2017010407302572000_25.17.3654.9","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/8.5.763"},{"key":"2017010407302572000_25.17.3654.10","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/10.2.137"},{"key":"2017010407302572000_25.17.3654.11","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/8.1.115"},{"key":"2017010407302572000_25.17.3654.12","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4547(19991115)58:4<515::AID-JNR5>3.0.CO;2-F"},{"key":"2017010407302572000_25.17.3654.13","doi-asserted-by":"publisher","DOI":"10.1038\/2510"},{"key":"2017010407302572000_25.17.3654.14","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.0857-08.2008"},{"key":"2017010407302572000_25.17.3654.15","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddg169"},{"key":"2017010407302572000_25.17.3654.16","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(00)80764-3"},{"key":"2017010407302572000_25.17.3654.17","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(00)81369-0"},{"key":"2017010407302572000_25.17.3654.18","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/8.3.397"},{"key":"2017010407302572000_25.17.3654.19","unstructured":"Brooks S.P. Dunnett S.B. (2015) Mouse Models of Huntington\u2019s Disease. In Nguyen P.H.H. Cenci A.M. (eds.), Behavioral Neurobiology of Huntington's Disease and Parkinson's Disease. Springer Berlin Heidelberg, Berlin, Heidelberg, pp. 101\u2013133."},{"key":"2017010407302572000_25.17.3654.20","doi-asserted-by":"publisher","DOI":"10.1038\/nrn3570"},{"key":"2017010407302572000_25.17.3654.21","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0049838"},{"key":"2017010407302572000_25.17.3654.22","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0050717"},{"key":"2017010407302572000_25.17.3654.23","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2006.12.010"},{"key":"2017010407302572000_25.17.3654.24","doi-asserted-by":"crossref","unstructured":"Mantovani S. Gordon R. Li R. Christie D.C. Kumar V. Woodruff T.M. (2016) Motor deficits associated with Huntington's disease occur in the absence of striatal degeneration in BACHD transgenic mice. Hum. Mol. Genet., pii: ddw050. [Epub ahead of print].","DOI":"10.1093\/hmg\/ddw050"},{"key":"2017010407302572000_25.17.3654.25","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/dds037"},{"key":"2017010407302572000_25.17.3654.26","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.4286-09.2009"},{"key":"2017010407302572000_25.17.3654.27","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2011.03.002"},{"key":"2017010407302572000_25.17.3654.28","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.93.23.13090"},{"key":"2017010407302572000_25.17.3654.29","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/28.21.e92"},{"key":"2017010407302572000_25.17.3654.30","doi-asserted-by":"publisher","DOI":"10.1002\/dvg.20310"},{"key":"2017010407302572000_25.17.3654.31","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2005.08.007"},{"key":"2017010407302572000_25.17.3654.32","doi-asserted-by":"crossref","first-page":"221","DOI":"10.3233\/JHD-129005","article-title":"Age-Dependent Resistance to Excitotoxicity in Htt CAG140 Mice and the Effect of Strain Background","volume":"1","author":"Strong","year":"2012","journal-title":"J. Huntingtons Dis"},{"key":"2017010407302572000_25.17.3654.33","doi-asserted-by":"crossref","unstructured":"Ghosh R. Tabrizi S. (2013) Clinical Aspects of Huntington's Disease. In Current Topics in Behavioral Neuroscience. Springer Berlin Heidelberg, pp. 1\u201329.","DOI":"10.1007\/7854_2013_238"},{"key":"2017010407302572000_25.17.3654.34","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2008.06.005"},{"key":"2017010407302572000_25.17.3654.35","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/dds397"},{"key":"2017010407302572000_25.17.3654.36","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2007.01.006"},{"key":"2017010407302572000_25.17.3654.37","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9568.2005.04324.x"},{"key":"2017010407302572000_25.17.3654.38","doi-asserted-by":"publisher","DOI":"10.1086\/318207"},{"key":"2017010407302572000_25.17.3654.39","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870110204"},{"key":"2017010407302572000_25.17.3654.40","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp006"},{"key":"2017010407302572000_25.17.3654.41","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.c3109"},{"key":"2017010407302572000_25.17.3654.42","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.0590-05.2005"},{"key":"2017010407302572000_25.17.3654.43","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-007-0306-6"},{"key":"2017010407302572000_25.17.3654.44","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(13)70088-7"},{"key":"2017010407302572000_25.17.3654.45","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":"2017010407302572000_25.17.3654.46","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200710158"},{"key":"2017010407302572000_25.17.3654.47","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddl013"},{"key":"2017010407302572000_25.17.3654.48","doi-asserted-by":"crossref","first-page":"4449","DOI":"10.1523\/JNEUROSCI.15-06-04449.1995","article-title":"Potassium currents responsible for inward and outward rectification in rat neostriatal spiny projection neurons","volume":"15","author":"Nisenbaum","year":"1995","journal-title":"J. Neurosci"},{"key":"2017010407302572000_25.17.3654.49","doi-asserted-by":"crossref","first-page":"2667","DOI":"10.1152\/jn.2001.86.6.2667","article-title":"Electrophysiological and Morphological Changes in Striatal Spiny Neurons in R6\/2 Huntington's Disease Transgenic Mice","volume":"86","author":"Klapstein","year":"2001","journal-title":"J. Neurophysiol"},{"key":"2017010407302572000_25.17.3654.50","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.3364-04.2005"},{"key":"2017010407302572000_25.17.3654.51","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/10.22.2515"},{"key":"2017010407302572000_25.17.3654.52","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1002\/wrna.17","article-title":"No-go decay: a quality control mechanism for RNA in translation","volume":"1","author":"Harigaya","year":"2010","journal-title":"Wiley Interdisciplinary Reviews - RNA"},{"key":"2017010407302572000_25.17.3654.53","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddv080"},{"key":"2017010407302572000_25.17.3654.54","doi-asserted-by":"publisher","DOI":"10.1016\/j.stem.2012.04.027"},{"key":"2017010407302572000_25.17.3654.55","doi-asserted-by":"publisher","DOI":"10.1016\/0896-6273(95)90106-X"},{"key":"2017010407302572000_25.17.3654.56","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1326-10-1"},{"key":"2017010407302572000_25.17.3654.57","doi-asserted-by":"crossref","first-page":"491","DOI":"10.3233\/JHD-130079","article-title":"Increased Steady-State Mutant Huntingtin mRNA in Huntington\u2019s Disease Brain","volume":"2","author":"Liu","year":"2014","journal-title":"J. Huntington's Dis"},{"key":"2017010407302572000_25.17.3654.58","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.94.8.4103"},{"key":"2017010407302572000_25.17.3654.59","doi-asserted-by":"publisher","DOI":"10.1152\/physrev.00041.2009"},{"key":"2017010407302572000_25.17.3654.60","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20428"},{"key":"2017010407302572000_25.17.3654.61","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199805000-00001"},{"key":"2017010407302572000_25.17.3654.62","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2011.03.018"},{"key":"2017010407302572000_25.17.3654.63","doi-asserted-by":"publisher","DOI":"10.1016\/0304-3940(91)90583-F"},{"key":"2017010407302572000_25.17.3654.64","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddm133"},{"key":"2017010407302572000_25.17.3654.65","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awg077"},{"key":"2017010407302572000_25.17.3654.66","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.88.6.2065"},{"key":"2017010407302572000_25.17.3654.67","doi-asserted-by":"publisher","DOI":"10.1016\/j.neurobiolaging.2012.08.006"},{"key":"2017010407302572000_25.17.3654.68","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-9568.2007.05307.x"},{"key":"2017010407302572000_25.17.3654.69","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1003930"},{"key":"2017010407302572000_25.17.3654.70","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1326-6-59"},{"key":"2017010407302572000_25.17.3654.71","doi-asserted-by":"crossref","first-page":"4510","DOI":"10.1093\/hmg\/ddu166","article-title":"Progressive axonal transport and synaptic protein changes correlate with behavioral and neuropathological abnormalities in the heterozygous Q175 KI mouse model of Huntington's disease","volume":"23","author":"Smith","year":"2014","journal-title":"Hum. Mol.Genet"},{"key":"2017010407302572000_25.17.3654.72","doi-asserted-by":"publisher","DOI":"10.1152\/jn.01056.2014"}],"container-title":["Human Molecular Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/hmg\/article\/2525826\/An-enhanced-Q175-knockin-mouse-model-of-Huntington","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,9,23]],"date-time":"2020-09-23T09:33:55Z","timestamp":1600853635000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/hmg\/article-lookup\/doi\/10.1093\/hmg\/ddw212"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,7,4]]},"references-count":72,"journal-issue":{"issue":"17","published-online":{"date-parts":[[2017,1,4]]},"published-print":{"date-parts":[[2016,9,1]]}},"alternative-id":["10.1093\/hmg\/ddw212"],"URL":"https:\/\/doi.org\/10.1093\/hmg\/ddw212","relation":{},"ISSN":["0964-6906","1460-2083"],"issn-type":[{"value":"0964-6906","type":"print"},{"value":"1460-2083","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,7,4]]}}}