{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T22:52:02Z","timestamp":1792191122245,"version":"4.4.0"},"reference-count":84,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T00:00:00Z","timestamp":1606780800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T00:00:00Z","timestamp":1606780800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2020,10,11]],"date-time":"2020-10-11T00:00:00Z","timestamp":1602374400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100005725","name":"CHDI Foundation, Inc.","doi-asserted-by":"publisher","award":["A11103"],"award-info":[{"award-number":["A11103"]}],"id":[{"id":"10.13039\/100005725","id-type":"DOI","asserted-by":"publisher"}]},{"name":"JPND National Funding Agencies"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurobiology of Disease"],"published-print":{"date-parts":[[2020,12]]},"DOI":"10.1016\/j.nbd.2020.105140","type":"journal-article","created":{"date-parts":[[2020,10,13]],"date-time":"2020-10-13T02:19:28Z","timestamp":1602555568000},"page":"105140","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":13,"special_numbering":"C","title":["RUES2 hESCs exhibit MGE-biased neuronal differentiation and muHTT-dependent defective specification hinting at SP1"],"prefix":"10.1016","volume":"146","author":[{"given":"Paola","family":"Conforti","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dario","family":"Besusso","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Brocchetti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilaria","family":"Campus","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Cappadona","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maura","family":"Galimberti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela","family":"Laporta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raffaele","family":"Iennaco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Riccardo L.","family":"Rossi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vittoria Bocchi","family":"Dickinson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Cattaneo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2020.105140_bb0005","doi-asserted-by":"crossref","DOI":"10.1093\/cercor\/bht254","article-title":"The early fetal development of human neocortical GABAergic interneurons","author":"Al-Jaberi","year":"2015","journal-title":"Cereb. Cortex"},{"key":"10.1016\/j.nbd.2020.105140_bb8002","doi-asserted-by":"crossref","unstructured":"Ballas, N., & Mandel, G. (2005). The many faces of REST oversee epigenetic programming of neuronal genes. In Current Opinion in Neurobiology. https:\/\/doi.org\/10.1016\/j.conb.2005.08.015.","DOI":"10.1016\/j.conb.2005.08.015"},{"key":"10.1016\/j.nbd.2020.105140_bb0015","doi-asserted-by":"crossref","DOI":"10.1093\/nar\/gkp335","article-title":"MEME suite: tools for motif discovery and searching","author":"Bailey","year":"2009","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.nbd.2020.105140_bb0020","doi-asserted-by":"crossref","DOI":"10.1126\/science.aax3338","article-title":"Huntington\u2019s disease alters human neurodevelopment","author":"Barnat","year":"2020","journal-title":"Science."},{"key":"10.1016\/j.nbd.2020.105140_bb0030","doi-asserted-by":"crossref","DOI":"10.1016\/j.stemcr.2020.03.018","article-title":"Stem cell-derived human striatal progenitors innervate striatal targets and alleviate sensorimotor deficit in a rat model of Huntington disease","author":"Besusso","year":"2020","journal-title":"Stem Cell Rep."},{"key":"10.1016\/j.nbd.2020.105140_bb0035","article-title":"JASPAR, the open access database of transcription factor-binding profiles: New content and tools in the 2008 update","author":"Bryne","year":"2008","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.nbd.2020.105140_bb0040","article-title":"Highly efficient neural conversion of human ES and iPS cells by dual inhibition of SMAD signaling","author":"Chambers","year":"2009","journal-title":"Nature Biotechnology"},{"key":"10.1016\/j.nbd.2020.105140_bb0045","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pbio.1000609","article-title":"Retinoic acid functions as a key gabaergic differentiation signal in the basal ganglia","author":"Chatzi","year":"2011","journal-title":"PLoS Biol."},{"key":"10.1016\/j.nbd.2020.105140_bb0050","doi-asserted-by":"crossref","DOI":"10.1016\/j.nbd.2005.11.001","article-title":"Decreased association of the transcription factor Sp1 with genes downregulated in Huntington's disease","author":"Chen-Plotkin","year":"2006","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2020.105140_bb0055","doi-asserted-by":"crossref","DOI":"10.1016\/j.stemcr.2016.09.003","article-title":"Targeted differentiation of regional ventral Neuroprogenitors and related neuronal subtypes from human pluripotent stem cells","author":"Chi","year":"2016","journal-title":"Stem Cell Rep."},{"key":"10.1016\/j.nbd.2020.105140_bb6001","doi-asserted-by":"crossref","DOI":"10.1016\/0092-8674(95)90298-8","article-title":"REST: A mammalian silencer protein that restricts sodium channel gene expression to neurons","author":"Chong","year":"1995","journal-title":"Cell."},{"key":"10.1016\/j.nbd.2020.105140_bb0070","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.1715865115","article-title":"Faulty neuronal determination and cell polarization are reverted by modulating HD early phenotypes","author":"Conforti","year":"2018","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2020.105140_bb0080","doi-asserted-by":"crossref","DOI":"10.1038\/nn.3668","article-title":"Mutant Huntingtin promotes autonomous microglia activation via myeloid lineage-determining factors","author":"Crotti","year":"2014","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.nbd.2020.105140_bb0085","doi-asserted-by":"crossref","DOI":"10.1152\/ajpheart.01230.2008","article-title":"Spl-dependent activation of KLF4 is required for PDGF-BB-induced phenotypic modulation of smooth muscle","author":"Deaton","year":"2009","journal-title":"Am. J. Physiol. Heart Circ. Physiol."},{"key":"10.1016\/j.nbd.2020.105140_bb0090","doi-asserted-by":"crossref","DOI":"10.1007\/s12015-013-9441-8","article-title":"Human pluripotent stem cell differentiation into authentic striatal projection neurons","author":"Delli Carri","year":"2013","journal-title":"Stem Cell Rev. Rep."},{"key":"10.1016\/j.nbd.2020.105140_bb0095","doi-asserted-by":"crossref","DOI":"10.1126\/science.1072613","article-title":"Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease","author":"Dunah","year":"2002","journal-title":"Science."},{"key":"10.1016\/j.nbd.2020.105140_bb0100","doi-asserted-by":"crossref","DOI":"10.1016\/j.neuron.2008.08.017","article-title":"A time and a place for Nkx2-1 in interneuron specification and migration","author":"Elias","year":"2008","journal-title":"Neuron."},{"key":"10.1016\/j.nbd.2020.105140_bb0105","doi-asserted-by":"crossref","DOI":"10.1101\/gad.1616208","article-title":"Human ES cell-derived neural rosettes reveal a functionally distinct early neural stem cell stage","author":"Elkabetz","year":"2008","journal-title":"Genes Dev."},{"key":"10.1016\/j.nbd.2020.105140_bb0110","doi-asserted-by":"crossref","DOI":"10.1038\/ng.677","article-title":"Mutations in GRIN2A and GRIN2B encoding regulatory subunits of NMDA receptors cause variable neurodevelopmental phenotypes","author":"Endele","year":"2010","journal-title":"Nat. Genet."},{"key":"10.1016\/j.nbd.2020.105140_bb0115","doi-asserted-by":"crossref","DOI":"10.1523\/ENEURO.0349-16.2017","article-title":"The isl1-lhx3 complex promotes motor neuron specification by activating transcriptional pathways that enhance its own expression and formation","author":"Erb","year":"2017","journal-title":"ENeuro."},{"key":"10.1016\/j.nbd.2020.105140_bb0120","doi-asserted-by":"crossref","DOI":"10.1385\/JMN\/31:02:139","article-title":"Decreased VIP and VPAC2 receptor expression in the biological clock of the R6\/2 Huntington's disease mouse","author":"Fahrenkrug","year":"2007","journal-title":"J. Mol. Neurosci."},{"key":"10.1016\/j.nbd.2020.105140_bb0125","doi-asserted-by":"crossref","DOI":"10.1007\/978-1-4939-7825-0_6","article-title":"Mouse models of Huntington's disease","author":"Farshim","year":"2018","journal-title":"Methods Mol. Biol."},{"key":"10.1016\/j.nbd.2020.105140_bb0130","doi-asserted-by":"crossref","DOI":"10.1016\/j.stem.2010.03.001","article-title":"Efficient derivation of functional floor plate tissue from human embryonic stem cells","author":"Fasano","year":"2010","journal-title":"Cell Stem Cell"},{"key":"10.1016\/j.nbd.2020.105140_bb0150","doi-asserted-by":"crossref","DOI":"10.1089\/scd.2012.0264","article-title":"Derivation and isolation of NKX2.1-positive basal forebrain progenitors from human embryonic stem cells","author":"Germain","year":"2013","journal-title":"Stem Cells Dev."},{"key":"10.1016\/j.nbd.2020.105140_bb0155","doi-asserted-by":"crossref","DOI":"10.1016\/j.neuron.2010.06.027","article-title":"Huntingtin is required for mitotic spindle orientation and mammalian neurogenesis","author":"Godin","year":"2010","journal-title":"Neuron."},{"key":"10.1016\/j.nbd.2020.105140_bb0165","doi-asserted-by":"crossref","DOI":"10.1016\/j.tibs.2006.06.009","article-title":"Huntington's disease: seeing the pathogenic process through a genetic lens","author":"Gusella","year":"2006","journal-title":"Trends Biochem. Sci."},{"key":"10.1016\/j.nbd.2020.105140_bb0170","doi-asserted-by":"crossref","DOI":"10.1038\/s41587-019-0237-5","article-title":"Self-organizing neuruloids model developmental aspects of Huntington's disease in the ectodermal compartment","author":"Haremaki","year":"2019","journal-title":"Nat. Biotechnol."},{"key":"10.1016\/j.nbd.2020.105140_bb0175","article-title":"Direct Interactions Between Gli3, Wnt8b and Fgfs Underlie Patterning of the Dorsal Telencephalon","author":"Hasenpusch-Theil","year":"2017","journal-title":"Cereb. Cortex (New York, N.Y. : 1991)"},{"key":"10.1016\/j.nbd.2020.105140_bb0181","article-title":"Induced pluripotent stem cells from patients with huntington\u2019s disease show CAG repeat expansion associated phenotypes","author":"HD iPSC Consortium","year":"2012","journal-title":"Cell Stem Cell"},{"key":"10.1016\/j.nbd.2020.105140_bb0180","article-title":"Developmental alterations in Huntington\u2019s disease neural cells and pharmacological rescue in cells and mice","author":"HD iPSC Consortium","year":"2017","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.nbd.2020.105140_bb0190","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.0910012107","article-title":"Neural differentiation of human induced pluripotent stem cells follows developmental principles but with variable potency","author":"Hu","year":"2010","journal-title":"Proceedings of the National Academy of Sciences of the United States of America"},{"key":"10.1016\/j.nbd.2020.105140_bb0200","doi-asserted-by":"crossref","DOI":"10.1016\/j.celrep.2012.04.009","article-title":"Generation of regionally specified neural progenitors and functional neurons from human embryonic stem cells under defined conditions","author":"Kirkeby","year":"2012","journal-title":"Cell Rep."},{"key":"10.1016\/j.nbd.2020.105140_bb0205","article-title":"Fusion protein Isl1-Lhx3 specifies motor neuron fate by inducing motor neuron genes and concomitantly suppressing the interneuron programs","author":"Lee","year":"2012","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2020.105140_bb0210","doi-asserted-by":"crossref","DOI":"10.1212\/WNL.0b013e3182648b65","article-title":"Measures of growth in children at risk for Huntington disease","author":"Lee","year":"2012","journal-title":"Neurology."},{"key":"10.1016\/j.nbd.2020.105140_bb0215","article-title":"CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion","author":"Lee","year":"2012","journal-title":"Neurology."},{"key":"10.1016\/j.nbd.2020.105140_bb0220","doi-asserted-by":"crossref","DOI":"10.1242\/dev.036624","article-title":"Coordination of sonic hedgehog and Wnt signaling determines ventral and dorsal telencephalic neuron types from human embryonic stem cells","author":"Li","year":"2009","journal-title":"Development."},{"key":"10.1016\/j.nbd.2020.105140_bb0230","doi-asserted-by":"crossref","DOI":"10.1093\/hmg\/10.2.137","article-title":"Neurological abnormalities in a knock-in mouse model of Huntington's disease","author":"Lin","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2020.105140_bb0235","article-title":"Directed differentiation of forebrain GABA interneurons from human pluripotent stem cells","author":"Liu","year":"2013","journal-title":"Nat. Protoc."},{"key":"10.1016\/j.nbd.2020.105140_bb0240","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0148680","article-title":"Dominant-negative effects of adult-onset huntingtin mutations alter the division of human embryonic stem cells-derived neural cells","author":"Lopes","year":"2016","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2020.105140_bb0245","series-title":"Animal Models for Huntington \u2019 S Disease","author":"Lucas","year":"2011"},{"issue":"5","key":"10.1016\/j.nbd.2020.105140_bb0250","doi-asserted-by":"crossref","first-page":"934","DOI":"10.1016\/j.stemcr.2019.04.007","article-title":"WNT\/NOTCH pathway is essential for the maintenance and expansion of human MGE progenitors","volume":"12","author":"Ma","year":"2019","journal-title":"Stem Cell Rep."},{"key":"10.1016\/j.nbd.2020.105140_bb0255","doi-asserted-by":"crossref","DOI":"10.1093\/nar\/gkm272","article-title":"STAMP: A web tool for exploring DNA-binding motif similarities","author":"Mahony","year":"2007","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.nbd.2020.105140_bb0260","doi-asserted-by":"crossref","DOI":"10.1038\/nrn3155","article-title":"Interneuron dysfunction in psychiatric disorders","author":"Mar\u00edn","year":"2012","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.nbd.2020.105140_bb0265","doi-asserted-by":"crossref","DOI":"10.1016\/j.stem.2013.04.008","article-title":"Directed differentiation and functional maturation of cortical interneurons from human embryonic stem cells","author":"Maroof","year":"2013","journal-title":"Cell Stem Cell"},{"key":"10.1016\/j.nbd.2020.105140_bb0275","doi-asserted-by":"crossref","DOI":"10.1093\/nar\/gkg108","article-title":"TRANSFAC\u00ae: transcriptional regulation, from patterns to profiles","author":"Matys","year":"2003","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.nbd.2020.105140_bb0285","doi-asserted-by":"crossref","DOI":"10.1016\/j.celrep.2018.09.076","article-title":"Human Huntington's disease iPSC-derived cortical neurons display altered Transcriptomics, morphology, and maturation","author":"Mehta","year":"2018","journal-title":"Cell Rep."},{"key":"10.1016\/j.nbd.2020.105140_bb0290","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.0912171106","article-title":"Impairment of developmental stem cell-mediated striatal neurogenesis and pluripotency genes in a knock-in model of Huntington's disease","author":"Molero","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2020.105140_bb0295","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.1603871113","article-title":"Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington's disease","author":"Molero","year":"2016","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2020.105140_bb0305","doi-asserted-by":"crossref","DOI":"10.1242\/dev.034439","article-title":"Differential effects of BMP signaling on parvalbumin and somatostatin interneuron differentiation","author":"Mukhopadhyay","year":"2009","journal-title":"Development."},{"key":"10.1016\/j.nbd.2020.105140_bb0310","doi-asserted-by":"crossref","DOI":"10.1016\/j.parkreldis.2018.02.033","article-title":"Cortical thickness, stance control, and arithmetic skill: An exploratory study in premanifest Huntington disease","author":"Nanetti","year":"2018","journal-title":"Parkinsonism Relat. Disord."},{"key":"10.1016\/j.nbd.2020.105140_bb0315","doi-asserted-by":"crossref","DOI":"10.1093\/nar\/gky317","article-title":"RSAT 2018: regulatory sequence analysis tools 20th anniversary","author":"Nguyen","year":"2018","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.nbd.2020.105140_bb0320","doi-asserted-by":"crossref","DOI":"10.1016\/j.stem.2013.04.005","article-title":"Functional maturation of hPSC-derived forebrain interneurons requires an extended timeline and mimics human neural development","author":"Nicholas","year":"2013","journal-title":"Cell Stem Cell"},{"key":"10.1016\/j.nbd.2020.105140_bb0325","article-title":"Cerebral cortex structure in prodromal Huntington disease","author":"Nopoulos","year":"2011","journal-title":"Neurobiology of Disease."},{"issue":"12","key":"10.1016\/j.nbd.2020.105140_bb0335","doi-asserted-by":"crossref","first-page":"1804","DOI":"10.1038\/nn.3860","article-title":"Molecular and functional definition of the developing human striatum","volume":"17","author":"Onorati","year":"2014","journal-title":"Nat. Neurosci."},{"issue":"9","key":"10.1016\/j.nbd.2020.105140_bb0340","doi-asserted-by":"crossref","first-page":"2494","DOI":"10.1016\/j.celrep.2019.02.008","article-title":"Unbiased profiling of isogenic Huntington disease hPSC-derived CNS and peripheral cells reveals strong cell-type specificity of CAG length effects","volume":"26","author":"Ooi","year":"2019","journal-title":"Cell Rep."},{"key":"10.1016\/j.nbd.2020.105140_bb0345","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1038\/nbt1383","article-title":"Marked differences in differentiation propensity among human embryonic stem cell lines","volume":"26","author":"Osafune","year":"2008","journal-title":"Nat. Biotechnol."},{"key":"10.1016\/j.nbd.2020.105140_bb0350","doi-asserted-by":"crossref","DOI":"10.1016\/j.stem.2018.11.010","article-title":"Human ESC-derived chimeric mouse models of Huntington's disease reveal cell-intrinsic defects in glial progenitor cell differentiation","author":"Osipovitch","year":"2019","journal-title":"Cell Stem Cell"},{"key":"10.1016\/j.nbd.2020.105140_bb0360","doi-asserted-by":"crossref","DOI":"10.1038\/nature07457","article-title":"Failure of neuronal homeostasis results in common neuropsychiatric phenotypes","author":"Ramocki","year":"2008","journal-title":"Nature."},{"key":"10.1016\/j.nbd.2020.105140_bb0365","doi-asserted-by":"crossref","first-page":"14467","DOI":"10.1523\/JNEUROSCI.1278-15.2015","article-title":"Retinoic acid receptor \u03b2 controls development of striatonigral projection neurons through FGF-dependent and meis1-dependent mechanisms","author":"Rataj-Baniowska","year":"2015","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2020.105140_bb0370","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0014311","article-title":"Transcriptional activation of REST by Sp1 in huntington's disease models","author":"Ravache","year":"2010","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2020.105140_bb0375","doi-asserted-by":"crossref","DOI":"10.1128\/MCB.01600-13","article-title":"CtBP and associated LSD1 are required for transcriptional activation by NeuroD1 in gastrointestinal endocrine cells","author":"Ray","year":"2014","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.nbd.2020.105140_bb0380","doi-asserted-by":"crossref","DOI":"10.1016\/j.stemcr.2015.11.005","article-title":"Genomic analysis reveals disruption of striatal neuronal development and therapeutic targets in human Huntington's disease neural stem cells","author":"Ring","year":"2015","journal-title":"Stem Cell Rep."},{"key":"10.1016\/j.nbd.2020.105140_bb0385","doi-asserted-by":"crossref","DOI":"10.1242\/dev.156844","article-title":"Chromosomal instability during neurogenesis in huntington's disease","author":"Ruzo","year":"2018","journal-title":"Development (Cambridge)."},{"key":"10.1016\/j.nbd.2020.105140_bb0390","doi-asserted-by":"crossref","DOI":"10.3174\/ajnr.A6168","article-title":"Widespread increased diffusivity reveals early cortical degeneration in Huntington disease","author":"Sampedro","year":"2019","journal-title":"Am. J. Neuroradiol."},{"issue":"5202","key":"10.1016\/j.nbd.2020.105140_bb6002","doi-asserted-by":"crossref","first-page":"1360","DOI":"10.1126\/science.7871435","article-title":"The neuron-restrictive silencer factor (NRSF): a coordinate repressor of multiple neuron-specific genes","volume":"267(5202)","author":"Schoenherr","year":"1995","journal-title":"Science"},{"key":"10.1016\/j.nbd.2020.105140_bb0395","doi-asserted-by":"crossref","DOI":"10.1128\/MCB.00335-15","article-title":"Critical roles of the LIM domains of Lhx3 in recruiting Coactivators to the motor neuron-specifying Isl1-Lhx3 complex","author":"Seo","year":"2015","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.nbd.2020.105140_bb0405","doi-asserted-by":"crossref","DOI":"10.1038\/nn.3041","article-title":"Human cerebral cortex development from pluripotent stem cells to functional excitatory synapses","author":"Shi","year":"2012","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.nbd.2020.105140_bb0410","doi-asserted-by":"crossref","DOI":"10.1038\/79139","article-title":"Expanded polyglutamine stretches interact with TAF(II)130, interfering with CREB-dependent transcription","author":"Shimohata","year":"2000","journal-title":"Nat. Genet."},{"key":"10.1016\/j.nbd.2020.105140_bb0415","doi-asserted-by":"crossref","DOI":"10.1038\/35000576","article-title":"Motor disorder in Huntington's disease begins as a dysfunction in error feedback control","author":"Smith","year":"2000","journal-title":"Nature."},{"key":"10.1016\/j.nbd.2020.105140_bb0420","doi-asserted-by":"crossref","DOI":"10.1016\/j.stemcr.2020.01.015","article-title":"Aberrant development corrected in adult-onset Huntington's disease iPSC-derived neuronal cultures via WNT Signaling modulation","author":"Smith-Geater","year":"2020","journal-title":"Stem Cell Rep."},{"key":"10.1016\/j.nbd.2020.105140_bb8000","doi-asserted-by":"crossref","unstructured":"Strano, A., Tuck, E., Stubbs, V. E., & Livesey, F. J. (2020). Variable Outcomes in Neural Differentiation of Human PSCs Arise from Intrinsic Differences in Developmental Signaling Pathways. Cell Reports. https:\/\/doi.org\/10.1016\/j.celrep.2020.107732.","DOI":"10.1016\/j.celrep.2020.107732"},{"key":"10.1016\/j.nbd.2020.105140_bb0425","doi-asserted-by":"crossref","DOI":"10.1016\/S0092-8674(02)00823-1","article-title":"LIM factor Lhx3 contributes to the specification of motor neuron and interneuron identity through cell-type-specific protein-protein interactions","author":"Thaler","year":"2002","journal-title":"Cell."},{"key":"10.1016\/j.nbd.2020.105140_bb0430","doi-asserted-by":"crossref","DOI":"10.1016\/j.neuron.2016.06.033","article-title":"GABAergic interneurons in the Neocortex: from cellular properties to circuits","author":"Tremblay","year":"2016","journal-title":"Neuron."},{"key":"10.1016\/j.nbd.2020.105140_bb0435","article-title":"Sp1 regulates human Huntingtin gene expression","author":"Wang","year":"2012","journal-title":"J. Mol. Neurosci."},{"key":"10.1016\/j.nbd.2020.105140_bb0440","doi-asserted-by":"crossref","DOI":"10.1038\/ng1297-404","article-title":"Huntington is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion","author":"White","year":"1997","journal-title":"Nat. Genet."},{"key":"10.1016\/j.nbd.2020.105140_bb0450","doi-asserted-by":"crossref","DOI":"10.1093\/hmg\/10.17.1829","article-title":"Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington's disease","author":"Wyttenbach","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2020.105140_bb0455","doi-asserted-by":"crossref","DOI":"10.1523\/JNEUROSCI.1637-10.2010","article-title":"BDNF overexpression in the forebrain rescues Huntington's disease phenotypes in YAC128 mice","author":"Xie","year":"2010","journal-title":"J. Neurosci."},{"issue":"3","key":"10.1016\/j.nbd.2020.105140_bb0460","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1016\/j.stemcr.2017.01.022","article-title":"Reversal of phenotypic abnormalities by CRISPR\/Cas9-mediated gene correction in Huntington disease patient-derived induced pluripotent stem cells","volume":"8","author":"Xu","year":"2017","journal-title":"Stem Cell Rep."},{"key":"10.1016\/j.nbd.2020.105140_bb0465","doi-asserted-by":"crossref","DOI":"10.1210\/en.2005-0970","article-title":"Two promoters mediate transcription from the human LHX3 gene: involvement of nuclear factor I and specificity protein 1","author":"Yaden","year":"2006","journal-title":"Endocrinology."},{"key":"10.1016\/j.nbd.2020.105140_bb0470","doi-asserted-by":"crossref","DOI":"10.1007\/978-1-60761-769-3","article-title":"Differentiation of neural precursors and dopaminergic neurons from human embryonic stem cells","author":"Zhang","year":"2010","journal-title":"Methods Mol. Biol."},{"key":"10.1016\/j.nbd.2020.105140_bb0475","doi-asserted-by":"crossref","DOI":"10.1161\/ATVBAHA.111.244962","article-title":"TGF-\u03b21 downregulates AT1 receptor expression via PKC-\u03b4-mediated Sp1 dissociation from KLF4 and smad-mediated PPAR-\u03b3 association with KLF4","author":"Zhang","year":"2012","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"10.1016\/j.nbd.2020.105140_bb8001","doi-asserted-by":"crossref","unstructured":"Zhang, L., Zhang, P., Wang, G., Zhang, H., Zhang, Y., Yu, Y., Zhang, M., Xiao, J., Crespo, P., Hell, J. W., Lin, L., Huganir, R. L., & Zhu, J. J. (2018). Ras and Rap Signal Bidirectional Synaptic Plasticity via Distinct Subcellular Microdomains. Neuron. https:\/\/doi.org\/10.1016\/j.neuron.2018.03.049.","DOI":"10.1016\/j.neuron.2018.03.049"},{"key":"10.1016\/j.nbd.2020.105140_bb0485","doi-asserted-by":"crossref","DOI":"10.1038\/nrneurol.2009.54","article-title":"Brain-derived neurotrophic factor in neurodegenerative diseases","author":"Zuccato","year":"2009","journal-title":"In Nature Reviews Neurology"},{"key":"10.1016\/j.nbd.2020.105140_bb0490","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0022966","article-title":"Brain-derived neurotrophic factor in patients with Huntington's disease","author":"Zuccato","year":"2011","journal-title":"PLoS One"}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996120304150?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996120304150?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T05:26:37Z","timestamp":1760851597000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0969996120304150"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,12]]},"references-count":84,"alternative-id":["S0969996120304150"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2020.105140","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.738856912.793593628","asserted-by":"object"}]},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2020,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"RUES2 hESCs exhibit MGE-biased neuronal differentiation and muHTT-dependent defective specification hinting at SP1","name":"articletitle","label":"Article Title"},{"value":"Neurobiology of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nbd.2020.105140","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 Published by Elsevier Inc.","name":"copyright","label":"Copyright"}],"article-number":"105140"}}