{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T02:52:08Z","timestamp":1793069528561,"version":"4.5.0"},"reference-count":138,"publisher":"Frontiers Media SA","funder":[{"DOI":"10.13039\/100005725","name":"CHDI Foundation","doi-asserted-by":"publisher","award":["A-7449"],"award-info":[{"award-number":["A-7449"]}],"id":[{"id":"10.13039\/100005725","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Hum. Neurosci."],"DOI":"10.3389\/fnhum.2016.00317","type":"journal-article","created":{"date-parts":[[2016,6,29]],"date-time":"2016-06-29T05:15:20Z","timestamp":1467177320000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":72,"title":["Corticostriatal Dysfunction in Huntington\u2019s Disease: The Basics"],"prefix":"10.3389","volume":"10","author":[{"given":"Kendra D.","family":"Bunner","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"George V.","family":"Rebec","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2016,6,28]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"425","DOI":"10.1002\/ana.410310412","article-title":"Preferential loss of striato-external pallidal projection neurons in presymptomatic Huntington\u2019s disease","volume":"31","author":"Albin","year":"1992","journal-title":"Ann. Neurol."},{"key":"B2","doi-asserted-by":"publisher","first-page":"366","DOI":"10.1016\/0166-2236(89)90074-X","article-title":"The functional anatomy of basal ganglia disorders","volume":"12","author":"Albin","year":"1989","journal-title":"Trends Neurosci."},{"key":"B3","doi-asserted-by":"publisher","first-page":"266","DOI":"10.1016\/0166-2236(90)90107-L","article-title":"Functional architecture of basal ganglia circuits: neural substrates of parallel processing","volume":"13","author":"Alexander","year":"1990","journal-title":"Trends Neurosci."},{"key":"B4","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1111\/j.1460-9568.2009.07047.x","article-title":"Dopamine modulation of excitatory currents in the striatum is dictated by the expression of D1 or D2 receptors and modified by endocannabinoids","volume":"31","author":"Andr\u00e9","year":"2010","journal-title":"Eur. J. Neurosci."},{"key":"B5","doi-asserted-by":"publisher","first-page":"46","DOI":"10.3389\/fnsys.2011.00046","article-title":"Altered balance of activity in the striatal direct and indirect pathways in mouse models of Huntington\u2019s disease","volume":"5","author":"Andr\u00e9","year":"2011","journal-title":"Front. Syst. Neurosci."},{"key":"B6","doi-asserted-by":"publisher","first-page":"716","DOI":"10.1002\/jnr.10272","article-title":"Striatal neurochemical changes in transgenic models of Huntington\u2019s disease","volume":"68","author":"Ariano","year":"2002","journal-title":"J. Neurosci. Res."},{"key":"B7","doi-asserted-by":"publisher","first-page":"440","DOI":"10.1097\/00005072-199704000-00013","article-title":"Changes of NMDA receptor subunit (NR1, NR2B) and glutamate transporter (GLT1) mRNA expression in Huntington\u2019s disease\u2013an in situ hybridization study","volume":"56","author":"Arzberger","year":"1997","journal-title":"J. Neuropathol. Exp. Neuro."},{"key":"B8","doi-asserted-by":"publisher","first-page":"252","DOI":"10.1212\/WNL.50.1.252","article-title":"Frontal lobe volume in patients with Huntington\u2019s disease","volume":"50","author":"Aylward","year":"1998","journal-title":"Neurology"},{"key":"B9","doi-asserted-by":"publisher","first-page":"15005","DOI":"10.1038\/nrdp.2015.5","article-title":"Huntington disease","volume":"1","author":"Bates","year":"2015","journal-title":"Nat. Rev. Dis. Primers"},{"key":"B10","doi-asserted-by":"publisher","first-page":"14845","DOI":"10.1073\/pnas.1009874107","article-title":"Distinct subclasses of medium spiny neurons differentially regulate striatal motor behaviors","volume":"107","author":"Bateup","year":"2010","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"B11","doi-asserted-by":"publisher","first-page":"168","DOI":"10.1038\/321168a0","article-title":"Replication of the neurochemical characteristics of Huntington\u2019s disease by quinolinic acid","volume":"321","author":"Beal","year":"1986","journal-title":"Nature"},{"key":"B12","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1038\/sj.bjp.0706949","article-title":"Transporters for L-glutamate: an update on their molecular pharmacology and pathological involvement","volume":"150","author":"Beart","year":"2007","journal-title":"Br. J. Pharmacol."},{"key":"B13","doi-asserted-by":"publisher","first-page":"6809","DOI":"10.1073\/pnas.120166397","article-title":"Severe deficiencies in dopamine signaling in presymptomatic Hungtinton\u2019s disease mice","volume":"97","author":"Bibb","year":"2000","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"B14","doi-asserted-by":"publisher","first-page":"61","DOI":"10.3389\/fnsys.2011.00061","article-title":"In vivo dopamine efflux is decreased in striatum of both fragment (R6\/2) and full-length (YAC128) transgenic mouse models of Huntington\u2019s disease","volume":"5","author":"Callahan","year":"2011","journal-title":"Front. Syst. Neurosci."},{"key":"B15","doi-asserted-by":"publisher","first-page":"9280","DOI":"10.1073\/pnas.1016190108","article-title":"Changes in striatal procedural memory coding correlate with learning deficits in a mouse model of Huntington disease","volume":"108","author":"Cayzac","year":"2011","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"B17","doi-asserted-by":"publisher","first-page":"671","DOI":"10.1111\/j.1460-9568.2008.06038.x","article-title":"Differential electrophysiological properties of dopamine D1 and D2 receptor-containing striatal medium-sized spiny neurons","volume":"27","author":"Cepeda","year":"2008","journal-title":"Eur. J. Neurosci."},{"key":"B18","doi-asserted-by":"publisher","first-page":"525","DOI":"10.1002\/jnr.1244","article-title":"NMDA receptor function in mouse models of Huntington disease","volume":"66","author":"Cepeda","year":"2001","journal-title":"J. Neurosci. Res."},{"key":"B19","doi-asserted-by":"publisher","first-page":"7393","DOI":"10.1523\/JNEUROSCI.2137-12.2013","article-title":"Multiple sources of striatal inhibition are differentially affected in Huntington\u2019s disease mouse models","volume":"33","author":"Cepeda","year":"2013","journal-title":"J. Neurosci."},{"key":"B16","doi-asserted-by":"publisher","first-page":"pe20","DOI":"10.1126\/stke.3332006pe20","article-title":"Where do you think you are going? The NMDA-D1 receptor trap","volume":"2006","author":"Cepeda","year":"2006","journal-title":"Sci. STKE"},{"key":"B20","doi-asserted-by":"publisher","first-page":"253","DOI":"10.1016\/j.pneurobio.2006.11.001","article-title":"The corticostriatal pathway in Huntington\u2019s disease","volume":"81","author":"Cepeda","year":"2007","journal-title":"Prog. Neurobiol."},{"key":"B21","doi-asserted-by":"publisher","first-page":"6480","DOI":"10.1073\/pnas.95.11.6480","article-title":"Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human Huntington\u2019s disease gene","volume":"95","author":"Cha","year":"1998","journal-title":"Proc. Natl. Acad. Sci. U S A"},{"key":"B22","doi-asserted-by":"publisher","first-page":"2179","DOI":"10.2147\/DDDT.S58470","article-title":"Transgenic animal models for study of the pathogenesis of Huntington\u2019s disease","volume":"9","author":"Chang","year":"2015","journal-title":"Drug Des. Devel. Ther."},{"key":"B23","doi-asserted-by":"publisher","first-page":"114","DOI":"10.3389\/fnins.2013.00114","article-title":"Dopamine imbalance in Huntington\u2019s disease: a mechanism for the lack of behavioral flexibility","volume":"7","author":"Chen","year":"2013","journal-title":"Front. Neurosci."},{"key":"B24","doi-asserted-by":"publisher","first-page":"198","DOI":"10.1016\/0304-3940(86)90397-6","article-title":"Reduced high-affinity glutamate uptake sites in the brains of patients with Huntington\u2019s disease","volume":"67","author":"Cross","year":"1986","journal-title":"Neurosci. Lett."},{"key":"B25","doi-asserted-by":"publisher","first-page":"200","DOI":"10.1002\/ana.410270217","article-title":"Degeneration of pyramidal projection neurons in Huntington\u2019s disease cortex","volume":"27","author":"Cudkowicz","year":"1990","journal-title":"Ann. Neurol."},{"key":"B26","doi-asserted-by":"publisher","first-page":"238","DOI":"10.1038\/nature11846","article-title":"Concurrent activation of striatal direct and indirect pathways during action initiation","volume":"494","author":"Cui","year":"2013","journal-title":"Nature"},{"key":"B27","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1159\/000375126","article-title":"Dysfunctional dopaminergic neurones in mouse models of Huntington\u2019s disease: a role for SK3 channels","volume":"15","author":"Dall\u00e9rac","year":"2015","journal-title":"Neurodegener. Dis."},{"key":"B28","doi-asserted-by":"publisher","first-page":"537","DOI":"10.1016\/s0092-8674(00)80513-9","article-title":"Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation","volume":"90","author":"Davies","year":"1997","journal-title":"Cell."},{"key":"B29","doi-asserted-by":"publisher","first-page":"281","DOI":"10.1016\/0166-2236(90)90110-v","article-title":"Primate models of movement disorders of basal ganglia origin","volume":"13","author":"DeLong","year":"1990","journal-title":"Trends Neurosci."},{"key":"B30","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1001\/archneur.64.1.20","article-title":"Circuits and circuit disorders of the basal ganglia","volume":"64","author":"DeLong","year":"2007","journal-title":"Arch. Neurol."},{"key":"B31","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1016\/j.jchemneu.2006.07.001","article-title":"Differential perikaryal localization in rats of D1 and D2 dopamine receptors on striatal projection neuron types identified by retrograde labeling","volume":"32","author":"Deng","year":"2006","journal-title":"J. Chem. Neuroanat."},{"key":"B32","doi-asserted-by":"publisher","first-page":"286","DOI":"10.1016\/0166-2236(90)90111-m","article-title":"Excitotoxic injury of the neostriatum: a model for Huntington\u2019s disease","volume":"13","author":"DiFiglia","year":"1990","journal-title":"Trends Neurosci."},{"key":"B33","doi-asserted-by":"publisher","first-page":"393","DOI":"10.1038\/nn.2286","article-title":"D2R striatopallidal neurons inhibit both locomotor and drug reward processes","volume":"12","author":"Durieus","year":"2009","journal-title":"Nat. Neurosci."},{"key":"B37","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/B978-0-12-407824-6.00008-2","article-title":"Huntington\u2019s disease and dementia: from transgenic models to molecular neuropathology","volume-title":"Diet and Nutrition in Dementia and Cognitive Decline","author":"Estrada-S\u00e1nchez","year":"2015a"},{"key":"B38","doi-asserted-by":"publisher","first-page":"4440","DOI":"10.1523\/JNEUROSCI.2812-14.2015","article-title":"Cortical efferents lacking mutant huntingtin improve striatal neuronal activity and behavior in a conditional mouse model of Huntington\u2019s disease","volume":"35","author":"Estrada-S\u00e1nchez","year":"2015b","journal-title":"J. Neurosci."},{"key":"B39","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1016\/j.arcmed.2007.11.011","article-title":"Excitotoxic neuronal death and pathogenesis of Huntington\u2019s disease","volume":"39","author":"Estrada-S\u00e1nchez","year":"2008","journal-title":"Arch. Med. Res."},{"key":"B35","doi-asserted-by":"publisher","first-page":"1156","DOI":"10.1007\/s11064-010-0168-5","article-title":"Glycolysis inhibition decreases the levels of glutamate transporters and enhances glutamate neurotoxicity in the R6\/2 Huntington\u2019s disease mice","volume":"35","author":"Estrada-S\u00e1nchez","year":"2010","journal-title":"Neurochem. Res."},{"key":"B34","doi-asserted-by":"publisher","first-page":"78","DOI":"10.1016\/j.nbd.2008.12.017","article-title":"Glutamate toxicity in the striatum of the R6\/2 Huntington\u2019s disease transgenic mice is age-dependent and correlates with decreased levels of glutamate transporters","volume":"34","author":"Estrada-S\u00e1nchez","year":"2009","journal-title":"Neurobio. Dis."},{"key":"B36","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1016\/j.baga.2012.04.029","article-title":"Corticostriatal dysfunction and glutamate transporter 1 (GLT1) in Huntington\u2019s disease: interactions between neurons and astrocytes","volume":"2","author":"Estrada-S\u00e1nchez","year":"2012","journal-title":"Basal Ganglia"},{"key":"B150","doi-asserted-by":"publisher","first-page":"19","DOI":"10.3389\/fncir.2013.00019","article-title":"Role of cerebral cortex in the neuropathology of Huntington\u2019s disease","volume":"7","author":"Estrada-S\u00e1nchez","year":"2013","journal-title":"Front. Neural Circuits"},{"key":"B40","doi-asserted-by":"publisher","first-page":"3053","DOI":"10.1093\/hmg\/ddq212","article-title":"In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington\u2019s disease subjects","volume":"19","author":"Faideau","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"B41","doi-asserted-by":"publisher","first-page":"272","DOI":"10.1016\/j.pneurobio.2006.11.003","article-title":"N-Methyle-D-aspartate (NMDA) receptor function and excitotoxicity in Huntington\u2019s disease","volume":"81","author":"Fan","year":"2007","journal-title":"Prog. Neurobiol."},{"key":"B42","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1006\/exnr.1993.1006","article-title":"Excitotoxin lesions in primates as a model for Huntington\u2019s disease: Histopathologic and neurochemical characterization","volume":"119","author":"Ferrante","year":"1993","journal-title":"Exp. Neurol."},{"key":"B43","doi-asserted-by":"publisher","first-page":"17","DOI":"10.3233\/JHD-2012-120009","article-title":"Functional differences between direct and indirect striatal output pathways in Huntington\u2019s disease","volume":"1","author":"Galvan","year":"2012","journal-title":"J. Huntingtons Dis."},{"key":"B44","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1111\/j.1471-4159.1992.tb09283.x","article-title":"Increased cerebrospinal fluid dopamineand 3,4-dihydroxyphenylacetic acid levels in Huntington\u2019s disease: evidence for an overactive dopaminergic brain transmission","volume":"58","author":"Garrett","year":"1992","journal-title":"J. Neurochem."},{"key":"B45","doi-asserted-by":"publisher","first-page":"77","DOI":"10.1016\/j.nbd.2004.05.008","article-title":"Adenosine and glutamate extracellular concentrations and mitogen-activated protein kinases in the striatum of Huntington transgenic mice. Selective antagonism of adenosine A2A receptors reduces transmitter outflow","volume":"17","author":"Gianfriddo","year":"2004","journal-title":"Neurobio. Dis."},{"key":"B46","doi-asserted-by":"publisher","first-page":"2223","DOI":"10.1523\/JNEUROSCI.4870-09.2010","article-title":"Distinct roles of GABAergic interneurons in the regulation of striatal output pathways","volume":"30","author":"Gittis","year":"2010","journal-title":"J. Neurosci."},{"key":"B47","doi-asserted-by":"publisher","first-page":"6182","DOI":"10.1523\/JNEUROSCI.0857-08.2008","article-title":"Full-length human mutant huntingtin with a stable polyglutamine repeat can elicit progressive and selective neuropathogenesis in BACHD mice","volume":"28","author":"Gray","year":"2008","journal-title":"J. Neurosci."},{"key":"B48","doi-asserted-by":"publisher","first-page":"2577","DOI":"10.1002\/mds.23914","article-title":"Local field potential oscillations of the globus pallidus in Huntington\u2019s disease","volume":"26","author":"Groiss","year":"2011","journal-title":"Mov. Disord."},{"key":"B49","doi-asserted-by":"publisher","first-page":"234","DOI":"10.1038\/306234a0","article-title":"A polymorphic DNA marker genetically linked to Huntington\u2019s disease","volume":"306","author":"Gusella","year":"1983","journal-title":"Nature"},{"key":"B50","doi-asserted-by":"crossref","first-page":"2369","DOI":"10.1523\/JNEUROSCI.20-06-02369.2000","article-title":"Striatonigrostriatal pathways in primates form an ascending spiral from the shell to the dorsolateral striatum","volume":"20","author":"Haber","year":"2000","journal-title":"J. Neurosci."},{"key":"B51","doi-asserted-by":"publisher","first-page":"614","DOI":"10.1016\/s0028-3932(02)00218-x","article-title":"Ideomotor limb apraxia in Huntington\u2019s disease: implications for corticostriate involvement","volume":"41","author":"Hamilton","year":"2003","journal-title":"Neuropsychologia"},{"key":"B52","doi-asserted-by":"publisher","first-page":"682","DOI":"10.1038\/nrn2911","article-title":"Synaptic versus extrasynaptic NMDA receptor signaling: implications for neurodegenerative disorders","volume":"11","author":"Hardingham","year":"2010","journal-title":"Nat. Rev. Neurosci."},{"key":"B53","first-page":"159","article-title":"The epidemiology of Huntington\u2019s disease","volume-title":"Huntington\u2019s Disease","author":"Harper","year":"2001","edition":"3rd Edn."},{"key":"B54","first-page":"113","article-title":"Huntington\u2019s disease: genetic and molecular studies","volume-title":"Huntington\u2019s Disease","author":"Harper","year":"2002","edition":"3rd Edn."},{"key":"B55","doi-asserted-by":"publisher","first-page":"232","DOI":"10.1007\/s11064-007-9463-1","article-title":"Glutamate uptake is reduced in prefrontal cortex in Huntington\u2019s disease","volume":"33","author":"Hassel","year":"2008","journal-title":"Neurochem. Res."},{"key":"B56","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1016\/0304-3940(91)90583-f","article-title":"Neuronal loss in layers V and VI of cerebral cortex in Huntington\u2019s disease","volume":"133","author":"Hedreen","year":"1991","journal-title":"Neurosci. Lett."},{"key":"B57","doi-asserted-by":"publisher","first-page":"e50717","DOI":"10.1371\/journal.pone.0050717","article-title":"Characterization of neurophysiological and behavioral changes, MRI brain volumetry and 1H MRS in zQ175 knock-in mouse model of Huntington\u2019s disease","volume":"7","author":"Heikkinen","year":"2012","journal-title":"PLoS One"},{"key":"B58","doi-asserted-by":"publisher","first-page":"320","DOI":"10.1007\/BF00310376","article-title":"Cortical and striatal neuron number in Huntington\u2019s disease","volume":"88","author":"Heinsen","year":"1994","journal-title":"Acta Neuropathol."},{"key":"B59","doi-asserted-by":"publisher","first-page":"280","DOI":"10.1016\/j.neuroscience.2008.08.041","article-title":"Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington\u2019s disease mice","volume":"157","author":"Hickey","year":"2008","journal-title":"Neuroscience"},{"key":"B60","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1046\/j.1471-4159.2002.00804.x","article-title":"The role of dopamine in motor symptoms in the R6\/2 transgenic mouse model of Huntington\u2019s disease","volume":"81","author":"Hickey","year":"2002","journal-title":"J. Neurochem."},{"key":"B61","doi-asserted-by":"publisher","first-page":"8986","DOI":"10.1523\/JNEUROSCI.1238-11.2011","article-title":"Behavioral and in vivo electrophysiological evidence for presymptomatic alteration of prefrontostriatal processing in the transgenic rat model for Huntington disease","volume":"31","author":"H\u00f6hn","year":"2011","journal-title":"J. Neurosci."},{"key":"B62","doi-asserted-by":"publisher","first-page":"e47026","DOI":"10.1371\/journal.pone.0047026","article-title":"Dysfunctional behavioral modulation of corticostriatal communication in the R6\/2 mouse model of Huntington\u2019s disease","volume":"7","author":"Hong","year":"2012","journal-title":"PLoS One"},{"key":"B63","doi-asserted-by":"crossref","DOI":"10.1093\/med\/9780195380538.001.0001","volume-title":"Introduction to Neuropharmacology.","author":"Iversen","year":"2009"},{"key":"B64","doi-asserted-by":"publisher","first-page":"910","DOI":"10.1097\/NEN.0b013e3181ee005d","article-title":"Hyperdopaminergic status in experimental Huntington disease","volume":"69","author":"Jahanshahi","year":"2010","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"B65","doi-asserted-by":"publisher","first-page":"737","DOI":"10.1111\/j.1471-4159.2006.03762.x","article-title":"Dopamine release is severely compromised in the R6\/2 mouse model of Huntington\u2019s disease","volume":"97","author":"Johnson","year":"2006","journal-title":"J. Neurochem."},{"key":"B66","doi-asserted-by":"publisher","first-page":"494","DOI":"10.1021\/cn500100r","article-title":"Astrocytes and Huntington\u2019s disease","volume":"5","author":"Khakh","year":"2014","journal-title":"ACS Chem. Neurosci."},{"key":"B67","doi-asserted-by":"publisher","first-page":"942","DOI":"10.1038\/nn.4043","article-title":"Diversity of astrocyte functions and phenotypes in neural circuits","volume":"18","author":"Khakh","year":"2015","journal-title":"Nat. Neurosci."},{"key":"B151","doi-asserted-by":"publisher","first-page":"386","DOI":"10.1002\/ana.410220318","article-title":"Elevated serotonin and reduced dopamine in subregionally divided Huntington\u2019s disease striatum","volume":"22","author":"Kish","year":"1987","journal-title":"Ann. Neurol."},{"key":"B68","doi-asserted-by":"crossref","first-page":"3594","DOI":"10.1523\/JNEUROSCI.19-09-03594.1999","article-title":"Striatal neuronal activity and responsiveness to dopamine and glutamate after selective blockade of D1 and D2 dopamine receptors in freely moving rats","volume":"19","author":"Kiyatkin","year":"1999","journal-title":"J. Neurosci."},{"key":"B69","doi-asserted-by":"publisher","first-page":"1185","DOI":"10.1016\/S0140-6736(70)90367-3","article-title":"Predictive test for Huntington\u2019s chorea","volume":"2","author":"Klawans","year":"1970","journal-title":"Lancet"},{"key":"B70","doi-asserted-by":"publisher","first-page":"622","DOI":"10.1038\/nature09159","article-title":"Regulation of parkinsonian motor behaviors by optogenetic control of basal ganglia circuity","volume":"466","author":"Kravitz","year":"2010","journal-title":"Nature"},{"key":"B72","doi-asserted-by":"publisher","first-page":"543","DOI":"10.1016\/j.neuron.2008.11.005","article-title":"Striatal plasticity and basal ganglia circuit function","volume":"60","author":"Kreitzer","year":"2008","journal-title":"Neuron"},{"key":"B73","first-page":"28","article-title":"Clinical neurology of Huntington\u2019s disease","volume-title":"Huntington\u2019s Disease","author":"Kremer","year":"2001","edition":"3rd Edn."},{"key":"B74","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/s1734-1140(10)70238-3","article-title":"Huntington\u2019s disease: pathogenesis to animal models","volume":"62","author":"Kumar","year":"2010","journal-title":"Pharmacol. Rep."},{"key":"B75","doi-asserted-by":"publisher","first-page":"192","DOI":"10.1016\/j.nbd.2013.06.002","article-title":"Enhanced Ca2+-dependent glutamate release from astrocytes of the BACHD Huntington\u2019s disease mouse model","volume":"58","author":"Lee","year":"2013","journal-title":"Neurobio. Dis."},{"key":"B76","doi-asserted-by":"publisher","first-page":"935","DOI":"10.1007\/s00415-004-0461-9","article-title":"Cognitive changes in patients with Huntington\u2019s disease (HD) and asymptomatic carriers of the HD mutation\u2013a longitudinal follow-up study","volume":"251","author":"Lemiere","year":"2004","journal-title":"J. Neurol."},{"key":"B152","doi-asserted-by":"publisher","first-page":"4552","DOI":"10.1523\/jneurosci.5865-09.2010","article-title":"Aberrant Rab11-dependent trafficking of the neuronal glutamate transporter EAAC1 causes oxidative stress and cell death in Huntington\u2019s disease","volume":"30","author":"Li","year":"2010","journal-title":"J. Neurosci."},{"key":"B77","doi-asserted-by":"publisher","first-page":"807","DOI":"10.1006\/nbdi.2001.0430","article-title":"Impaired glutamate uptake in the R6 Huntington\u2019s disease transgenic mice","volume":"8","author":"Lievens","year":"2001","journal-title":"Neurobiol. Dis."},{"key":"B78","doi-asserted-by":"publisher","first-page":"443","DOI":"10.1038\/nn1654","article-title":"FACS-array profiling of striatal projection neuron subtypes in juvenile and adult mouse brains","volume":"9","author":"Lobo","year":"2006","journal-title":"Nat. Neurosci."},{"key":"B79","doi-asserted-by":"publisher","first-page":"3857","DOI":"10.1523\/JNEUROSCI.5027-04.2005","article-title":"Feedforward inhibition of projection neurons by fast-spiking GABA interneurons in the rat striatum in vivo","volume":"25","author":"Mallet","year":"2005","journal-title":"J. Neurosci."},{"key":"B80","doi-asserted-by":"publisher","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":"B81","doi-asserted-by":"publisher","first-page":"217","DOI":"10.1002\/glia.20027","article-title":"Role of glial amino acid transporters in synaptic transmission and brain energetics","volume":"47","author":"Marcaggi","year":"2004","journal-title":"Glia"},{"key":"B82","doi-asserted-by":"publisher","first-page":"503","DOI":"10.1016\/0306-4522(89)90128-0","article-title":"The organization of the projection from the cerebral cortex to the striatum in the rat","volume":"29","author":"McGeorge","year":"1989","journal-title":"Neuroscience"},{"key":"B87","doi-asserted-by":"publisher","first-page":"e49838","DOI":"10.1371\/journal.pone.0049838","article-title":"Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington\u2019s disease: zQ175","volume":"7","author":"Menalled","year":"2012","journal-title":"PLoS One"},{"key":"B83","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1002\/cne.10776","article-title":"Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington\u2019s disease with 140 CAG repeats","volume":"465","author":"Menalled","year":"2003","journal-title":"J. Comp. Neurol."},{"key":"B84","doi-asserted-by":"crossref","first-page":"8266","DOI":"10.1523\/JNEUROSCI.22-18-08266.2002","article-title":"Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington\u2019s disease knock-in mice","volume":"22","author":"Menalled","year":"2002","journal-title":"J. Neurosci."},{"key":"B86","doi-asserted-by":"publisher","first-page":"328","DOI":"10.1006\/exnr.1999.7327","article-title":"Decrease in striatal encephalin mRNA in mouse models of Huntington\u2019s disease","volume":"162","author":"Menalled","year":"2000","journal-title":"Exp. Neurol."},{"key":"B88","doi-asserted-by":"publisher","first-page":"629","DOI":"10.1111\/j.1471-4159.2012.07691.x","article-title":"Up-regulation of GLT1 reverses the deficit in cortically evoked striatal ascorbate efflux in the R6\/2 mouse model of Huntington\u2019s disease","volume":"121","author":"Miller","year":"2012","journal-title":"J. Neurochem."},{"key":"B89","doi-asserted-by":"publisher","first-page":"329","DOI":"10.1016\/j.neuroscience.2008.02.004","article-title":"Up-regulation of GLT1 expression increases glutamate uptake and attenuates the Huntington\u2019s disease phenotype in the R6\/2 mouse","volume":"153","author":"Miller","year":"2008","journal-title":"Neuroscience"},{"key":"B90","doi-asserted-by":"publisher","first-page":"26","DOI":"10.3389\/fnsys.2011.00026","article-title":"Dyregulated neuronal activity patterns implicates corticostriatal circuit dysfunction in multiple rodent models of Huntington\u2019s disease","volume":"5","author":"Miller","year":"2011","journal-title":"Front. Syst. Neurosci."},{"key":"B92","doi-asserted-by":"publisher","first-page":"178","DOI":"10.1016\/j.neuron.2010.01.008","article-title":"Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington\u2019s disease mice","volume":"65","author":"Milnerwood","year":"2010","journal-title":"Neuron"},{"key":"B91","doi-asserted-by":"publisher","first-page":"513","DOI":"10.1016\/j.tins.2010.08.002","article-title":"Early synaptic pathophysiology in neurodegeneration: insights from Huntington\u2019s disease","volume":"33","author":"Milnerwood","year":"2010","journal-title":"Trends Neurosci."},{"key":"B93","doi-asserted-by":"crossref","first-page":"5115","DOI":"10.1523\/JNEUROSCI.20-13-05115.2000","article-title":"Abnormal synaptic plasticity and impaired spatial congnition in mice transgenic for Exon 1 of the human Huntington\u2019s disease mutation","volume":"20","author":"Murphy","year":"2000","journal-title":"J. Neurosci."},{"key":"B94","doi-asserted-by":"publisher","first-page":"1049","DOI":"10.1016\/j.neuroscience.2003.08.053","article-title":"Molecular and behavioral analysis of the R6\/1 Huntington\u2019s disease transgenic mouse","volume":"122","author":"Naver","year":"2003","journal-title":"Neuroscience"},{"key":"B95","doi-asserted-by":"publisher","first-page":"206","DOI":"10.1046\/j.0953-816x.2000.01379.x","article-title":"Altered striatal amino acid neurotransmitter release monitored using microdialysis in R6\/1 Huntington transgenic mice","volume":"13","author":"NicNiocaill","year":"2001","journal-title":"Eur. J. Neurosci."},{"key":"B96","doi-asserted-by":"publisher","first-page":"1475","DOI":"10.1093\/hmg\/2.9.1475","article-title":"Trinucleotide repeat elongation in the Huntingtin gene in Huntington disease patients from 71 Danish families","volume":"2","author":"N\u00f8rrem\u00f8lle","year":"1993","journal-title":"Hum. Mol. Genet."},{"key":"B97","doi-asserted-by":"publisher","first-page":"1407","DOI":"10.1038\/nm.2056","article-title":"Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin","volume":"15","author":"Okamoto","year":"2009","journal-title":"Nat. Med."},{"key":"B98","doi-asserted-by":"publisher","first-page":"755","DOI":"10.1111\/j.1471-4159.2009.06501.x","article-title":"Dyregulation of intracellular dopamine stores revealed in the R6\/2 mouse striatum","volume":"112","author":"Ortiz","year":"2010","journal-title":"J. Neurochem."},{"key":"B100","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1016\/j.neulet.2011.01.036","article-title":"Impaired dopamine release and uptake in R6\/1 Huntington\u2019s disease model mice","volume":"492","author":"Ortiz","year":"2011","journal-title":"Neurosci. Lett."},{"key":"B99","doi-asserted-by":"publisher","first-page":"148","DOI":"10.1016\/j.brainres.2012.02.042","article-title":"Motor function and dopamine release measurements in transgenic Huntington\u2019s disease model rats","volume":"1450","author":"Ortiz","year":"2012","journal-title":"Brain Res."},{"key":"B101","doi-asserted-by":"publisher","first-page":"202","DOI":"10.1002\/cne.20925","article-title":"Single-axon tracing study of corticostriatal projections arising from primary motor cortex in primates","volume":"496","author":"Parent","year":"2006","journal-title":"J. Comp. Neurol."},{"key":"B102","doi-asserted-by":"publisher","first-page":"2219","DOI":"10.1093\/hmg\/dds037","article-title":"Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice","volume":"21","author":"Pouladi","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"B103","first-page":"129","article-title":"Neurotransmitter and their receptors","volume-title":"Neuroscience","author":"Purves","year":"2008","edition":"4th Edn."},{"key":"B104","doi-asserted-by":"publisher","first-page":"8158","DOI":"10.1007\/978-1-4615-0715-4_40","article-title":"Synaptic convergence of motor and somatosensory cortical afferents onto GABAergic interneurons in the rat striatum","volume":"22","author":"Ramanathan","year":"2002","journal-title":"J. Neurosci."},{"key":"B105","doi-asserted-by":"publisher","first-page":"144","DOI":"10.1159\/000443738","article-title":"The prevalence of Huntington\u2019s disease","volume":"46","author":"Rawlins","year":"2016","journal-title":"Neuroepidemiology"},{"key":"B106","doi-asserted-by":"publisher","first-page":"327","DOI":"10.1016\/j.neuroscience.2005.08.062","article-title":"Hyperactive striatal neurons in symptomatic Huntington R6\/2 mice: variations with behavioral state and repeated ascorbate treatment","volume":"137","author":"Rebec","year":"2006","journal-title":"Neuroscience"},{"key":"B108","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1016\/B978-0-12-374767-9.00018-4","article-title":"Organization of corticostriatal projection neuron types","volume-title":"Handbook of Basal Ganglia Structure and Function","author":"Reiner","year":"2010"},{"key":"B109","doi-asserted-by":"publisher","first-page":"420","DOI":"10.1002\/cne.10541","article-title":"Differential morphology of pyramidal tract-type and intratelencephalically projecting-type corticostriatal neurons and their intrastriatal terminals in rats","volume":"457","author":"Reiner","year":"2003","journal-title":"J. Comp. Neurol."},{"key":"B107","doi-asserted-by":"publisher","first-page":"1691","DOI":"10.1002\/mds.25624","article-title":"Striatal parvalbuminergic neurons are lost in Huntington\u2019s disease: implications for dystonia","volume":"28","author":"Reiner","year":"2013","journal-title":"Mov. Disord."},{"key":"B110","doi-asserted-by":"publisher","first-page":"121","DOI":"10.1016\/0304-3940(91)90448-3","article-title":"Heterogeneous dopamine receptor changes in early and late Huntington\u2019s disease","volume":"132","author":"Richfield","year":"1991","journal-title":"Neurosci. Lett."},{"key":"B111","doi-asserted-by":"publisher","first-page":"1025","DOI":"10.1212\/wnl.57.6.1025","article-title":"Striatal volume loss in HD as measured by MRI and the influence of CAG repeat","volume":"57","author":"Rosas","year":"2001","journal-title":"Neurology"},{"key":"B112","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1038\/nature03180","article-title":"Beta-lactam antibiotics offer neuroprotection by increasing glutamate transporter expression","volume":"433","author":"Rothstein","year":"2005","journal-title":"Nature"},{"key":"B113","first-page":"16","article-title":"Phenotypic characterization of individuals with 30\u201340 CAG repeats in the Huntington\u2019s disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36\u201339 repeats","volume":"59","author":"Rubinsztein","year":"1996","journal-title":"Am. J. Hum. Genet."},{"key":"B114","doi-asserted-by":"publisher","first-page":"397","DOI":"10.1016\/0306-4522(94)00427-7","article-title":"Evidence for a preferential loss of encephalin immunoreactivity in the external globus pallidus in low grade Huntington\u2019s disease using high resolution image analysis","volume":"64","author":"Sapp","year":"1995","journal-title":"Neuroscience"},{"key":"B115","doi-asserted-by":"publisher","first-page":"62","DOI":"10.1186\/1423-0127-17-62","article-title":"Ceftriaxone-induced up-regulation of cortical and striatal GLT1 in the R6\/2 model of Huntington\u2019s disease","volume":"17","author":"Sari","year":"2010","journal-title":"J. Biomed. Sci."},{"key":"B116","doi-asserted-by":"publisher","first-page":"392","DOI":"10.1016\/j.nbd.2005.08.001","article-title":"Striatal neuronal apoptosis is preferentially enhanced by NMDA receptor activation in YAC transgenic mouse model of Huntington disease","volume":"21","author":"Shehadeh","year":"2006","journal-title":"Neurobiol. Dis."},{"key":"B117","doi-asserted-by":"publisher","first-page":"278","DOI":"10.1038\/nrn3469","article-title":"Corticostriatal connectivity and its role in disease","volume":"14","author":"Shepherd","year":"2013","journal-title":"Nat. Rev. Neurosci."},{"key":"B118","doi-asserted-by":"publisher","first-page":"R443","DOI":"10.1016\/j.cub.2007.03.044","article-title":"Structure and function of the cerebral cortex","volume":"17","author":"Shipp","year":"2007","journal-title":"Curr. Biol."},{"key":"B119","doi-asserted-by":"publisher","first-page":"1555","DOI":"10.1093\/hmg\/ddg169","article-title":"Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease","volume":"12","author":"Slow","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"B120","doi-asserted-by":"publisher","first-page":"1117","DOI":"10.1212\/wnl.41.7.1117","article-title":"Morphometric analysis of the prefrontal cortex in Huntington\u2019s disease","volume":"41","author":"Sotrel","year":"1991","journal-title":"Neurology"},{"key":"B121","doi-asserted-by":"publisher","first-page":"118","DOI":"10.1016\/s0169-328x(02)00216-4","article-title":"Differential D1 and D2 receptor-mediated effects on immediate early gene induction in a transgenic mouse model of Huntington\u2019s disease","volume":"102","author":"Spektor","year":"2002","journal-title":"Brain Res. Mol. Brain Res."},{"key":"B122","doi-asserted-by":"publisher","first-page":"354","DOI":"10.1002\/cne.20680","article-title":"Chronology of behavioral symptoms and neuropathological sequela in R6\/2 Huntington\u2019s disease transgenic mice","volume":"490","author":"Stack","year":"2005","journal-title":"J. Comp. Neurol."},{"key":"B123","doi-asserted-by":"publisher","first-page":"228","DOI":"10.1016\/j.tins.2007.03.008","article-title":"D1 and D2 dopamine-receptor modulation of striatal glutamatergic signaling in striatal medium spiny neurons","volume":"30","author":"Surmeier","year":"2007","journal-title":"Trends Neurosci."},{"key":"B124","doi-asserted-by":"publisher","first-page":"329","DOI":"10.1002\/syn.1089","article-title":"Vesicular neurotransmitter transporters in Huntington\u2019s disease: initial observations and comparison with traditional synaptic markers","volume":"41","author":"Suzuki","year":"2001","journal-title":"Synapse"},{"key":"B125","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1016\/j.brainres.2007.03.053","article-title":"Membrane properties and synaptic connectivity of fast-spiking interneurons in rat ventral striatum","volume":"1152","author":"Taverna","year":"2007","journal-title":"Brain Res."},{"key":"B126","doi-asserted-by":"publisher","first-page":"1535","DOI":"10.1093\/hmg\/2.10.1535","article-title":"Molecular analysis of juvenile Huntington disease: the major influence on (CAG) repeat length is the sex of the affected parent","volume":"2","author":"Telenius","year":"1993","journal-title":"Hum. Mol. Genet."},{"key":"B127","doi-asserted-by":"publisher","first-page":"662","DOI":"10.1016\/j.tins.2004.10.005","article-title":"GABAergic microcircuits in the neostriatum","volume":"27","author":"Tepper","year":"2004","journal-title":"Trends Neurosci."},{"key":"B128","doi-asserted-by":"publisher","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-e","article-title":"A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington\u2019s disease chromosomes","volume":"72","author":"The Huntington\u2019s Disease Collaborative Research Group","year":"1993","journal-title":"Cell"},{"key":"B129","doi-asserted-by":"publisher","first-page":"694","DOI":"10.1038\/nn.3691","article-title":"Astrocyte Kir4.1 ion channel deficits contribute to neuronal dysfunction in Huntington\u2019s disease model mice","volume":"17","author":"Tong","year":"2014","journal-title":"Nat. Neurosci."},{"key":"B130","doi-asserted-by":"publisher","first-page":"226","DOI":"10.1111\/j.1468-1331.2008.02390.x","article-title":"Changes in striatal dopamine D2 receptor binding in preclinical Huntington\u2019s disease","volume":"16","author":"van Oostrom","year":"2009","journal-title":"Eur. J. Neurol."},{"key":"B131","doi-asserted-by":"publisher","first-page":"3823","DOI":"10.1093\/hmg\/ddi407","article-title":"Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease","volume":"14","author":"Van Raamsdonk","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"B133","doi-asserted-by":"publisher","first-page":"369","DOI":"10.1097\/00005072-199805000-00001","article-title":"Huntington disease","volume":"57","author":"Vonsattel","year":"1998","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"B134","doi-asserted-by":"publisher","first-page":"8973","DOI":"10.1523\/JNEUROSCI.2804-08.2008","article-title":"Altered information processing in the prefrontal cortex of Huntington\u2019s disease mouse models","volume":"28","author":"Walker","year":"2008","journal-title":"J. Neurosci."},{"key":"B135","doi-asserted-by":"publisher","first-page":"379","DOI":"10.1016\/j.nbd.2011.04.009","article-title":"Reduced expression of conditioned fear in the R6\/2 mouse model of Huntington\u2019s disease is related to abnormal activity in prelimbic cortex","volume":"43","author":"Walker","year":"2011","journal-title":"Neurobiol. Dis."},{"key":"B136","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1002\/ana.410400110","article-title":"Striatal D1 and D2 dopamine receptor loss in asymptomatic mutation carriers of Huntington\u2019s disease","volume":"40","author":"Weeks","year":"1996","journal-title":"Ann. Neurol."},{"key":"B137","doi-asserted-by":"publisher","first-page":"466","DOI":"10.1016\/0006-8993(82)90385-7","article-title":"Ornithine aminotransferase in Huntington\u2019s disease","volume":"231","author":"Wong","year":"1982","journal-title":"Brain Res."},{"key":"B138","doi-asserted-by":"publisher","first-page":"905","DOI":"10.1152\/physrev.00041.2009","article-title":"Molecular mechanisms and potential therapeutic targets in Huntington\u2019s disease","volume":"90","author":"Zuccato","year":"2010","journal-title":"Physiol. Rev."}],"container-title":["Frontiers in Human Neuroscience"],"original-title":[],"deposited":{"date-parts":[[2019,9,10]],"date-time":"2019-09-10T06:23:47Z","timestamp":1568096627000},"score":1,"resource":{"primary":{"URL":"http:\/\/journal.frontiersin.org\/Article\/10.3389\/fnhum.2016.00317\/abstract"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,6,28]]},"references-count":138,"URL":"https:\/\/doi.org\/10.3389\/fnhum.2016.00317","relation":{},"ISSN":["1662-5161"],"issn-type":[{"value":"1662-5161","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,6,28]]}}}