{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T22:52:03Z","timestamp":1792191123468,"version":"4.4.0"},"reference-count":159,"publisher":"Springer Science and Business Media LLC","issue":"4","license":[{"start":{"date-parts":[[2017,5,11]],"date-time":"2017-05-11T00:00:00Z","timestamp":1494460800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100004281","name":"Narodowe Centrum Nauki","doi-asserted-by":"publisher","award":["2013\/10\/E\/NZ4\/00621"],"award-info":[{"award-number":["2013\/10\/E\/NZ4\/00621"]}],"id":[{"id":"10.13039\/501100004281","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Polish Ministry of Science and Higher Education","award":["KNOW program (01\/KNOW2\/2014)"],"award-info":[{"award-number":["KNOW program (01\/KNOW2\/2014)"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Mol Neurobiol"],"published-print":{"date-parts":[[2018,4]]},"DOI":"10.1007\/s12035-017-0477-7","type":"journal-article","created":{"date-parts":[[2017,5,11]],"date-time":"2017-05-11T12:56:00Z","timestamp":1494507360000},"page":"3351-3371","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":90,"title":["Huntington Disease as a Neurodevelopmental Disorder and Early Signs of the Disease in Stem Cells"],"prefix":"10.1007","volume":"55","author":[{"given":"Kalina","family":"Wiatr","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wojciech J.","family":"Szlachcic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Trzeciak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marek","family":"Figlerowicz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maciej","family":"Figiel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2017,5,11]]},"reference":[{"key":"477_CR1","doi-asserted-by":"publisher","first-page":"78","DOI":"10.3389\/fnagi.2014.00078","volume":"6","author":"JS Paulsen","year":"2014","unstructured":"Paulsen JS, Long JD, Johnson HJ et al (2014) Clinical and Biomarker Changes in Premanifest Huntington Disease Show Trial Feasibility: A Decade of the PREDICT-HD Study. Front Aging Neurosci 6:78. doi: 10.3389\/fnagi.2014.00078","journal-title":"Front Aging Neurosci"},{"key":"477_CR2","doi-asserted-by":"publisher","DOI":"10.1111\/cge.12893","author":"KA Quaid","year":"2016","unstructured":"Quaid KA, Eberly SW, Kayson-Rubin E et al (2016) Factors related to genetic testing in adults at risk for Huntington disease: the prospective Huntington atrisk observational study (PHAROS). Clin Genet. doi: 10.1111\/cge.12893","journal-title":"Clin Genet."},{"key":"477_CR3","doi-asserted-by":"publisher","first-page":"147","DOI":"10.3389\/fneur.2016.00147","volume":"7","author":"JA Ciarochi","year":"2016","unstructured":"Ciarochi JA, Calhoun VD, Lourens S et al (2016) Patterns of Co-Occurring Gray Matter Concentration Loss across the Huntington Disease Prodrome. Front Neurol 7:147. doi: 10.3389\/fneur.2016.00147","journal-title":"Front Neurol"},{"key":"477_CR4","doi-asserted-by":"publisher","first-page":"184","DOI":"10.1176\/appi.ajp.2015.14121551","volume":"173","author":"EA Epping","year":"2016","unstructured":"Epping EA, Kim J-I, Craufurd D et al (2016) Longitudinal Psychiatric Symptoms in Prodromal Huntington\u2019s Disease: A Decade of Data. Am J Psychiatry 173:184\u2013192. doi: 10.1176\/appi.ajp.2015.14121551","journal-title":"Am J Psychiatry"},{"key":"477_CR5","doi-asserted-by":"publisher","first-page":"2332","DOI":"10.1093\/brain\/awv145","volume":"138","author":"DL Harrington","year":"2015","unstructured":"Harrington DL, Rubinov M, Durgerian S et al (2015) Network topology and functional connectivity disturbances precede the onset of Huntington\u2019s disease. Brain J Neurol 138:2332\u20132346. doi: 10.1093\/brain\/awv145","journal-title":"Brain J Neurol"},{"key":"477_CR6","doi-asserted-by":"publisher","first-page":"1584","DOI":"10.1038\/ejhg.2015.11","volume":"23","author":"J-I Kim","year":"2015","unstructured":"Kim J-I, Long JD, Mills JA et al (2015) Performance of the 12-item WHODAS 2.0 in prodromal Huntington disease. Eur J Hum Genet EJHG 23:1584\u20131587. doi: 10.1038\/ejhg.2015.11","journal-title":"Eur J Hum Genet EJHG"},{"key":"477_CR7","doi-asserted-by":"publisher","first-page":"874","DOI":"10.1136\/jnnp.2007.128728","volume":"79","author":"JS Paulsen","year":"2008","unstructured":"Paulsen JS, Langbehn DR, Stout JC et al (2008) Detection of Huntington\u2019s disease decades before diagnosis: the Predict-HD study. J Neurol Neurosurg Psychiatry 79:874\u2013880. doi: 10.1136\/jnnp.2007.128728","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"477_CR8","doi-asserted-by":"publisher","first-page":"255","DOI":"10.1037\/neu0000102","volume":"29","author":"JK Williams","year":"2015","unstructured":"Williams JK, Kim J-I, Downing N et al (2015) Everyday cognition in prodromal Huntington disease. Neuropsychology 29:255\u2013267. doi: 10.1037\/neu0000102","journal-title":"Neuropsychology"},{"key":"477_CR9","doi-asserted-by":"publisher","first-page":"2856","DOI":"10.1093\/hmg\/ddl224","volume":"15","author":"DM Cummings","year":"2006","unstructured":"Cummings DM, Milnerwood AJ, Dall\u00e9rac GM et al (2006) Aberrant cortical synaptic plasticity and dopaminergic dysfunction in a mouse model of Huntington\u2019s disease. Hum Mol Genet 15:2856\u20132868. doi: 10.1093\/hmg\/ddl224","journal-title":"Hum Mol Genet"},{"key":"477_CR10","doi-asserted-by":"publisher","first-page":"965","DOI":"10.1093\/hmg\/ddl013","volume":"15","author":"A Hodges","year":"2006","unstructured":"Hodges A, Strand AD, Aragaki AK et al (2006) Regional and cellular gene expression changes in human Huntington\u2019s disease brain. Hum Mol Genet 15:965\u2013977. doi: 10.1093\/hmg\/ddl013","journal-title":"Hum Mol Genet"},{"key":"477_CR11","doi-asserted-by":"publisher","first-page":"1845","DOI":"10.1093\/hmg\/ddm133","volume":"16","author":"A Kuhn","year":"2007","unstructured":"Kuhn A, Goldstein DR, Hodges A et al (2007) Mutant huntingtin\u2019s effects on striatal gene expression in mice recapitulate changes observed in human Huntington\u2019s disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage. Hum Mol Genet 16:1845\u20131861. doi: 10.1093\/hmg\/ddm133","journal-title":"Hum Mol Genet"},{"key":"477_CR12","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0143563","volume":"10","author":"A Labadorf","year":"2015","unstructured":"Labadorf A, Hoss AG, Lagomarsino V et al (2015) RNA Sequence Analysis of Human Huntington Disease Brain Reveals an Extensive Increase in Inflammatory and Developmental Gene Expression. PloS One 10:e0143563. doi: 10.1371\/journal.pone.0143563","journal-title":"PloS One"},{"key":"477_CR13","doi-asserted-by":"publisher","first-page":"1690","DOI":"10.1093\/hmg\/ddl092","volume":"15","author":"AJ Milnerwood","year":"2006","unstructured":"Milnerwood AJ, Cummings DM, Dall\u00e9rac GM et al (2006) Early development of aberrant synaptic plasticity in a mouse model of Huntington\u2019s disease. Hum Mol Genet 15:1690\u20131703. doi: 10.1093\/hmg\/ddl092","journal-title":"Hum Mol Genet"},{"key":"477_CR14","doi-asserted-by":"publisher","first-page":"959","DOI":"10.1016\/j.biopsych.2008.12.026","volume":"65","author":"S Schippling","year":"2009","unstructured":"Schippling S, Schneider SA, Bhatia KP et al (2009) Abnormal motor cortex excitability in preclinical and very early Huntington\u2019s disease. Biol Psychiatry 65:959\u2013965. doi: 10.1016\/j.biopsych.2008.12.026","journal-title":"Biol Psychiatry"},{"key":"477_CR15","doi-asserted-by":"publisher","first-page":"668","DOI":"10.1212\/WNL.0b013e3182648b65","volume":"79","author":"JK Lee","year":"2012","unstructured":"Lee JK, Mathews K, Schlaggar B et al (2012) Measures of growth in children at risk for Huntington disease. Neurology 79:668\u2013674. doi: 10.1212\/WNL.0b013e3182648b65","journal-title":"Neurology"},{"key":"477_CR16","doi-asserted-by":"publisher","first-page":"231","DOI":"10.3233\/JHD-150152","volume":"4","author":"A Tereshchenko","year":"2015","unstructured":"Tereshchenko A, McHugh M, Lee JK et al (2015) Abnormal Weight and Body Mass Index in Children with Juvenile Huntington\u2019s Disease. J Huntingt Dis 4:231\u2013238. doi: 10.3233\/JHD-150152","journal-title":"J Huntingt Dis"},{"key":"477_CR17","doi-asserted-by":"publisher","first-page":"1042","DOI":"10.1038\/ejhg.2013.15","volume":"21","author":"V Eatough","year":"2013","unstructured":"Eatough V, Santini H, Eiser C et al (2013) The personal experience of parenting a child with Juvenile Huntington\u2019s Disease: perceptions across Europe. Eur J Hum Genet 21:1042\u20131048. doi: 10.1038\/ejhg.2013.15","journal-title":"Eur J Hum Genet"},{"key":"477_CR18","doi-asserted-by":"publisher","first-page":"208","DOI":"10.1016\/j.mad.2005.09.012","volume":"127","author":"F Squitieri","year":"2006","unstructured":"Squitieri F, Frati L, Ciarmiello A et al (2006) Juvenile Huntington\u2019s disease: does a dosage-effect pathogenic mechanism differ from the classical adult disease? Mech Ageing Dev 127:208\u2013212. doi: 10.1016\/j.mad.2005.09.012","journal-title":"Mech Ageing Dev"},{"key":"477_CR19","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.mcn.2008.06.004","volume":"40","author":"MT Lorincz","year":"2009","unstructured":"Lorincz MT, Zawistowski VA (2009) Expanded CAG repeats in the murine Huntington\u2019s disease gene increases neuronal differentiation of embryonic and neural stem cells. Mol Cell Neurosci 40:1\u201313. doi: 10.1016\/j.mcn.2008.06.004","journal-title":"Mol Cell Neurosci"},{"key":"477_CR20","doi-asserted-by":"publisher","first-page":"37","DOI":"10.3389\/fncel.2013.00037","volume":"7","author":"JC Niclis","year":"2013","unstructured":"Niclis JC, Pinar A, Haynes JM et al (2013) Characterization of forebrain neurons derived from late-onset Huntington\u2019s disease human embryonic stem cell lines. Front Cell Neurosci 7:37. doi: 10.3389\/fncel.2013.00037","journal-title":"Front Cell Neurosci"},{"key":"477_CR21","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0072698","volume":"8","author":"GD Nguyen","year":"2013","unstructured":"Nguyen GD, Molero AE, Gokhan S, Mehler MF (2013) Functions of huntingtin in germ layer specification and organogenesis. PloS One 8:e72698. doi: 10.1371\/journal.pone.0072698","journal-title":"PloS One"},{"key":"477_CR22","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1016\/j.nbd.2011.12.032","volume":"46","author":"V Castiglioni","year":"2012","unstructured":"Castiglioni V, Onorati M, Rochon C, Cattaneo E (2012) Induced pluripotent stem cell lines from Huntington\u2019s disease mice undergo neuronal differentiation while showing alterations in the lysosomal pathway. Neurobiol Dis 46:30\u201340. doi: 10.1016\/j.nbd.2011.12.032","journal-title":"Neurobiol Dis"},{"key":"477_CR23","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1016\/j.nbd.2011.12.042","volume":"46","author":"S Camnasio","year":"2012","unstructured":"Camnasio S, Delli Carri A, Lombardo A et al (2012) The first reported generation of several induced pluripotent stem cell lines from homozygous and heterozygous Huntington\u2019s disease patients demonstrates mutation related enhanced lysosomal activity. Neurobiol Dis 46:41\u201351. doi: 10.1016\/j.nbd.2011.12.042","journal-title":"Neurobiol Dis"},{"key":"477_CR24","doi-asserted-by":"publisher","first-page":"253","DOI":"10.1016\/j.stem.2012.04.026","volume":"11","author":"MC An","year":"2012","unstructured":"An MC, Zhang N, Scott G et al (2012) Genetic correction of Huntington\u2019s disease phenotypes in induced pluripotent stem cells. Cell Stem Cell 11:253\u2013263. doi: 10.1016\/j.stem.2012.04.026","journal-title":"Cell Stem Cell"},{"key":"477_CR25","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1186\/s13024-016-0092-5","volume":"11","author":"ED Nekrasov","year":"2016","unstructured":"Nekrasov ED, Vigont VA, Klyushnikov SA et al (2016) Manifestation of Huntington\u2019s disease pathology in human induced pluripotent stem cell-derived neurons. Mol Neurodegener 11:27. doi: 10.1186\/s13024-016-0092-5","journal-title":"Mol Neurodegener"},{"key":"477_CR26","doi-asserted-by":"publisher","first-page":"264","DOI":"10.1016\/j.stem.2012.04.027","volume":"11","author":"HD iPSC Consortium","year":"2012","unstructured":"HD iPSC Consortium (2012) Induced pluripotent stem cells from patients with Huntington\u2019s disease show CAG-repeat-expansion-associated phenotypes. Cell Stem Cell 11:264\u2013278. doi: 10.1016\/j.stem.2012.04.027","journal-title":"Cell Stem Cell"},{"key":"477_CR27","doi-asserted-by":"publisher","first-page":"160","DOI":"10.1016\/j.nbd.2012.10.015","volume":"50","author":"P Conforti","year":"2013","unstructured":"Conforti P, Camnasio S, Mutti C et al (2013) Lack of huntingtin promotes neural stem cells differentiation into glial cells while neurons expressing huntingtin with expanded polyglutamine tracts undergo cell death. Neurobiol Dis 50:160\u2013170. doi: 10.1016\/j.nbd.2012.10.015","journal-title":"Neurobiol Dis"},{"key":"477_CR28","doi-asserted-by":"publisher","first-page":"70","DOI":"10.1016\/j.mcn.2012.03.011","volume":"50","author":"JJ Ritch","year":"2012","unstructured":"Ritch JJ, Valencia A, Alexander J et al (2012) Multiple phenotypes in Huntington disease mouse neural stem cells. Mol Cell Neurosci 50:70\u201381. doi: 10.1016\/j.mcn.2012.03.011","journal-title":"Mol Cell Neurosci"},{"key":"477_CR29","doi-asserted-by":"publisher","first-page":"e64368","DOI":"10.1371\/journal.pone.0064368","volume":"8","author":"GD Nguyen","year":"2013","unstructured":"Nguyen GD, Gokhan S, Molero AE, Mehler MF (2013) Selective roles of normal and mutant huntingtin in neural induction and early neurogenesis. PloS One 8:e64368. doi: 10.1371\/journal.pone.0064368","journal-title":"PloS One"},{"key":"477_CR30","doi-asserted-by":"publisher","first-page":"3257","DOI":"10.1093\/hmg\/ddv080","volume":"24","author":"VB Mattis","year":"2015","unstructured":"Mattis VB, Tom C, Akimov S et al (2015) HD iPSC-derived neural progenitors accumulate in culture and are susceptible to BDNF withdrawal due to glutamate toxicity. Hum Mol Genet 24:3257\u20133271. doi: 10.1093\/hmg\/ddv080","journal-title":"Hum Mol Genet"},{"key":"477_CR31","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1016\/S1472-6483(10)60053-3","volume":"19","author":"JC Niclis","year":"2009","unstructured":"Niclis JC, Trounson AO, Dottori M et al (2009) Human embryonic stem cell models of Huntington disease. Reprod Biomed Online 19:106\u2013113","journal-title":"Reprod Biomed Online"},{"key":"477_CR32","doi-asserted-by":"publisher","first-page":"2442","DOI":"10.1093\/hmg\/ddv006","volume":"24","author":"M Biagioli","year":"2015","unstructured":"Biagioli M, Ferrari F, Mendenhall EM et al (2015) Htt CAG repeat expansion confers pleiotropic gains of mutant huntingtin function in chromatin regulation. Hum Mol Genet 24:2442\u20132457. doi: 10.1093\/hmg\/ddv006","journal-title":"Hum Mol Genet"},{"key":"477_CR33","doi-asserted-by":"publisher","first-page":"6066","DOI":"10.1093\/hmg\/ddv318","volume":"24","author":"F-L Chiu","year":"2015","unstructured":"Chiu F-L, Lin J-T, Chuang C-Y et al (2015) Elucidating the role of the A2A adenosine receptor in neurodegeneration using neurons derived from Huntington\u2019s disease iPSCs. Hum Mol Genet 24:6066\u20136079. doi: 10.1093\/hmg\/ddv318","journal-title":"Hum Mol Genet"},{"key":"477_CR34","doi-asserted-by":"publisher","first-page":"5648","DOI":"10.1021\/pr500649m","volume":"13","author":"LR McQuade","year":"2014","unstructured":"McQuade LR, Balachandran A, Scott HA et al (2014) Proteomics of Huntington\u2019s disease-affected human embryonic stem cells reveals an evolving pathology involving mitochondrial dysfunction and metabolic disturbances. J Proteome Res 13:5648\u20135659. doi: 10.1021\/pr500649m","journal-title":"J Proteome Res"},{"key":"477_CR35","doi-asserted-by":"publisher","first-page":"176","DOI":"10.1093\/hmg\/ddq456","volume":"20","author":"A Seriola","year":"2011","unstructured":"Seriola A, Spits C, Simard JP et al (2011) Huntington\u2019s and myotonic dystrophy hESCs: down-regulated trinucleotide repeat instability and mismatch repair machinery expression upon differentiation. Hum Mol Genet 20:176\u2013185. doi: 10.1093\/hmg\/ddq456","journal-title":"Hum Mol Genet"},{"key":"477_CR36","doi-asserted-by":"publisher","first-page":"1264","DOI":"10.1016\/j.scr.2013.08.010","volume":"11","author":"I Jonson","year":"2013","unstructured":"Jonson I, Ougland R, Klungland A, Larsen E (2013) Oxidative stress causes DNA triplet expansion in Huntington\u2019s disease mouse embryonic stem cells. Stem Cell Res 11:1264\u20131271. doi: 10.1016\/j.scr.2013.08.010","journal-title":"Stem Cell Res"},{"key":"477_CR37","doi-asserted-by":"publisher","first-page":"1820","DOI":"10.1096\/fj.12-219220","volume":"27","author":"B Lu","year":"2013","unstructured":"Lu B, Palacino J (2013) A novel human embryonic stem cell-derived Huntington\u2019s disease neuronal model exhibits mutant huntingtin (mHTT) aggregates and soluble mHTT-dependent neurodegeneration. FASEB J Off Publ Fed Am Soc Exp Biol 27:1820\u20131829. doi: 10.1096\/fj.12-219220","journal-title":"FASEB J Off Publ Fed Am Soc Exp Biol"},{"key":"477_CR38","doi-asserted-by":"publisher","first-page":"2054","DOI":"10.1002\/stem.1135","volume":"30","author":"I Jeon","year":"2012","unstructured":"Jeon I, Lee N, Li J-Y et al (2012) Neuronal properties, in vivo effects, and pathology of a Huntington\u2019s disease patient-derived induced pluripotent stem cells. Stem Cells Dayt Ohio 30:2054\u20132062. doi: 10.1002\/stem.1135","journal-title":"Stem Cells Dayt Ohio"},{"key":"477_CR39","doi-asserted-by":"publisher","first-page":"585","DOI":"10.1016\/j.stemcr.2014.07.011","volume":"3","author":"RL Carter","year":"2014","unstructured":"Carter RL, Chen Y, Kunkanjanawan T et al (2014) Reversal of cellular phenotypes in neural cells derived from Huntington\u2019s disease monkey-induced pluripotent stem cells. Stem Cell Rep 3:585\u2013593. doi: 10.1016\/j.stemcr.2014.07.011","journal-title":"Stem Cell Rep"},{"key":"477_CR40","doi-asserted-by":"publisher","first-page":"2846","DOI":"10.1093\/hmg\/ddr195","volume":"20","author":"JC Jacobsen","year":"2011","unstructured":"Jacobsen JC, Gregory GC, Woda JM et al (2011) HD CAG-correlated gene expression changes support a simple dominant gain of function. Hum Mol Genet 20:2846\u20132860. doi: 10.1093\/hmg\/ddr195","journal-title":"Hum Mol Genet"},{"key":"477_CR41","doi-asserted-by":"publisher","first-page":"230","DOI":"10.1016\/j.ydbio.2014.04.005","volume":"391","author":"I Ismailoglu","year":"2014","unstructured":"Ismailoglu I, Chen Q, Popowski M et al (2014) Huntingtin protein is essential for mitochondrial metabolism, bioenergetics and structure in murine embryonic stem cells. Dev Biol 391:230\u2013240. doi: 10.1016\/j.ydbio.2014.04.005","journal-title":"Dev Biol"},{"key":"477_CR42","doi-asserted-by":"publisher","first-page":"1023","DOI":"10.1016\/j.stemcr.2015.11.005","volume":"5","author":"KL Ring","year":"2015","unstructured":"Ring KL, An MC, Zhang N et al (2015) Genomic Analysis Reveals Disruption of Striatal Neuronal Development and Therapeutic Targets in Human Huntington\u2019s Disease Neural Stem Cells. Stem Cell Rep 5:1023\u20131038. doi: 10.1016\/j.stemcr.2015.11.005","journal-title":"Stem Cell Rep"},{"key":"477_CR43","doi-asserted-by":"publisher","first-page":"5371","DOI":"10.1172\/JCI70911","volume":"123","author":"X Guo","year":"2013","unstructured":"Guo X, Disatnik M-H, Monbureau M et al (2013) Inhibition of mitochondrial fragmentation diminishes Huntington\u2019s disease-associated neurodegeneration. J Clin Invest 123:5371\u20135388. doi: 10.1172\/JCI70911","journal-title":"J Clin Invest"},{"key":"477_CR44","doi-asserted-by":"publisher","first-page":"1816","DOI":"10.1002\/stem.1430","volume":"31","author":"J Charbord","year":"2013","unstructured":"Charbord J, Poydenot P, Bonnefond C et al (2013) High throughput screening for inhibitors of REST in neural derivatives of human embryonic stem cells reveals a chemical compound that promotes expression of neuronal genes. Stem Cells Dayt Ohio 31:1816\u20131828. doi: 10.1002\/stem.1430","journal-title":"Stem Cells Dayt Ohio"},{"key":"477_CR45","doi-asserted-by":"publisher","unstructured":"Lu X-H, Mattis VB, Wang N, et al (2014) Targeting ATM ameliorates mutant Huntingtin toxicity in cell and animal models of Huntington\u2019s disease. Sci Transl Med 6:268ra178. doi: 10.1126\/scitranslmed.3010523","DOI":"10.1126\/scitranslmed.3010523"},{"key":"477_CR46","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.05449","author":"Y Yao","year":"2015","unstructured":"Yao Y, Cui X, Al-Ramahi I et al (2015) A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity. eLife. doi: 10.7554\/eLife.05449","journal-title":"eLife."},{"key":"477_CR47","doi-asserted-by":"publisher","first-page":"359","DOI":"10.1042\/BJ20111495","volume":"446","author":"J-I Chae","year":"2012","unstructured":"Chae J-I, Kim D-W, Lee N et al (2012) Quantitative proteomic analysis of induced pluripotent stem cells derived from a human Huntington\u2019s disease patient. Biochem J 446:359\u2013371. doi: 10.1042\/BJ20111495","journal-title":"Biochem J"},{"key":"477_CR48","doi-asserted-by":"publisher","unstructured":"Szlachcic WJ, Switonski PM, Krzyzosiak WJ, et al (2015) Huntington disease iPSCs show early molecular changes in intracellular signaling, the expression of oxidative stress proteins and the p53 pathway. Dis Model Mech 8:1047\u2013 1057. doi: 10.1242\/dmm.019406","DOI":"10.1242\/dmm.019406"},{"key":"477_CR49","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1126\/science.7618107","volume":"269","author":"MP Duyao","year":"1995","unstructured":"Duyao MP, Auerbach AB, Ryan A et al (1995) Inactivation of the mouse Huntington\u2019s disease gene homolog Hdh. Science 269:407\u2013410","journal-title":"Science"},{"key":"477_CR50","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1016\/0092-8674(95)90542-1","volume":"81","author":"J Nasir","year":"1995","unstructured":"Nasir J, Floresco SB, O\u2019Kusky JR et al (1995) Targeted disruption of the Huntington\u2019s disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes. Cell 81:811\u2013823","journal-title":"Cell"},{"key":"477_CR51","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1038\/ng1095-155","volume":"11","author":"S Zeitlin","year":"1995","unstructured":"Zeitlin S, Liu JP, Chapman DL et al (1995) Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington\u2019s disease gene homologue. Nat Genet 11:155\u2013163. doi: 10.1038\/ng1095-155","journal-title":"Nat Genet"},{"key":"477_CR52","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1186\/1471-213X-5-17","volume":"5","author":"JM Woda","year":"2005","unstructured":"Woda JM, Calzonetti T, Hilditch-Maguire P et al (2005) Inactivation of the Huntington\u2019s disease gene (Hdh) impairs anterior streak formation and early patterning of the mouse embryo. BMC Dev Biol 5:17. doi: 10.1186\/1471-213X-5-17","journal-title":"BMC Dev Biol"},{"key":"477_CR53","doi-asserted-by":"crossref","first-page":"2515","DOI":"10.1093\/hmg\/10.22.2515","volume":"10","author":"W Auerbach","year":"2001","unstructured":"Auerbach W, Hurlbert MS, Hilditch-Maguire P et al (2001) The HD mutation causes progressive lethal neurological disease in mice expressing reduced levels of huntingtin. Hum Mol Genet 10:2515\u20132523","journal-title":"Hum Mol Genet"},{"key":"477_CR54","doi-asserted-by":"publisher","first-page":"404","DOI":"10.1038\/ng1297-404","volume":"17","author":"JK White","year":"1997","unstructured":"White JK, Auerbach W, Duyao MP et al (1997) Huntingtin is required for neurogenesis and is not impaired by the Huntington\u2019s disease CAG expansion. Nat Genet 17:404\u2013410. doi: 10.1038\/ng1297-404","journal-title":"Nat Genet"},{"key":"477_CR55","doi-asserted-by":"publisher","first-page":"144","DOI":"10.1016\/j.nbd.2016.09.006","volume":"96","author":"EE Arteaga-Bracho","year":"2016","unstructured":"Arteaga-Bracho EE, Gulinello M, Winchester ML et al (2016) Postnatal and adult consequences of loss of huntingtin during development: Implications for Huntington\u2019s disease. Neurobiol Dis 96:144\u2013155. doi: 10.1016\/j.nbd.2016.09.006","journal-title":"Neurobiol Dis"},{"key":"477_CR56","doi-asserted-by":"publisher","first-page":"5736","DOI":"10.1073\/pnas.1603871113","volume":"113","author":"AE Molero","year":"2016","unstructured":"Molero AE, Arteaga-Bracho EE, Chen CH et al (2016) Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington\u2019s disease. Proc Natl Acad Sci U S A 113:5736\u20135741. doi: 10.1073\/pnas.1603871113","journal-title":"Proc Natl Acad Sci U S A"},{"key":"477_CR57","doi-asserted-by":"publisher","first-page":"300","DOI":"10.1038\/81593","volume":"26","author":"I Dragatsis","year":"2000","unstructured":"Dragatsis I, Levine MS, Zeitlin S (2000) Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice. Nat Genet 26:300\u2013306. doi: 10.1038\/81593","journal-title":"Nat Genet"},{"key":"477_CR58","doi-asserted-by":"publisher","first-page":"1031","DOI":"10.1016\/j.neuron.2012.05.009","volume":"74","author":"HB Kordasiewicz","year":"2012","unstructured":"Kordasiewicz HB, Stanek LM, Wancewicz EV et al (2012) Sustained therapeutic reversal of Huntington\u2019s disease by transient repression of huntingtin synthesis. Neuron 74:1031\u20131044. doi: 10.1016\/j.neuron.2012.05.009","journal-title":"Neuron"},{"key":"477_CR59","doi-asserted-by":"publisher","first-page":"3359","DOI":"10.1073\/pnas.1524575113","volume":"113","author":"G Wang","year":"2016","unstructured":"Wang G, Liu X, Gaertig MA et al (2016) Ablation of huntingtin in adult neurons is nondeleterious but its depletion in young mice causes acute pancreatitis. Proc Natl Acad Sci U S A 113:3359\u20133364. doi: 10.1073\/pnas.1524575113","journal-title":"Proc Natl Acad Sci U S A"},{"key":"477_CR60","doi-asserted-by":"publisher","first-page":"142","DOI":"10.1093\/hmg\/ddn324","volume":"18","author":"P Dietrich","year":"2009","unstructured":"Dietrich P, Shanmugasundaram R, Shuyu E, Dragatsis I (2009) Congenital hydrocephalus associated with abnormal subcommissural organ in mice lacking huntingtin in Wnt1 cell lineages. Hum Mol Genet 18:142\u2013150. doi: 10.1093\/hmg\/ddn324","journal-title":"Hum Mol Genet"},{"key":"477_CR61","doi-asserted-by":"publisher","first-page":"e1006083","DOI":"10.1371\/journal.pgen.1006083","volume":"12","author":"X Liu","year":"2016","unstructured":"Liu X, Wang C-E, Hong Y et al (2016) N-terminal Huntingtin Knock-In Mice: Implications of Removing the N-terminal Region of Huntingtin for Therapy. PLoS Genet 12:e1006083. doi: 10.1371\/journal.pgen.1006083","journal-title":"PLoS Genet"},{"key":"477_CR62","doi-asserted-by":"publisher","first-page":"71","DOI":"10.3233\/JHD-2012-120016","volume":"1","author":"M Neveklovska","year":"2012","unstructured":"Neveklovska M, Clabough EBD, Steffan JS, Zeitlin SO (2012) Deletion of the huntingtin proline-rich region does not significantly affect normal huntingtin function in mice. J Huntingt Dis 1:71\u201387. doi: 10.3233\/JHD-2012-120016","journal-title":"J Huntingt Dis"},{"key":"477_CR63","doi-asserted-by":"publisher","first-page":"e1000838","DOI":"10.1371\/journal.pgen.1000838","volume":"6","author":"S Zheng","year":"2010","unstructured":"Zheng S, Clabough EBD, Sarkar S et al (2010) Deletion of the huntingtin polyglutamine stretch enhances neuronal autophagy and longevity in mice. PLoS Genet 6:e1000838. doi: 10.1371\/journal.pgen.1000838","journal-title":"PLoS Genet"},{"key":"477_CR64","doi-asserted-by":"publisher","first-page":"577","DOI":"10.1007\/s12015-012-9349-8","volume":"8","author":"L Grabel","year":"2012","unstructured":"Grabel L (2012) Developmental origin of neural stem cells: the glial cell that could. Stem Cell Rev 8:577\u2013585. doi: 10.1007\/s12015-012-9349-8","journal-title":"Stem Cell Rev"},{"key":"477_CR65","doi-asserted-by":"publisher","first-page":"713","DOI":"10.1038\/nn.3080","volume":"15","author":"V Lo Sardo","year":"2012","unstructured":"Lo Sardo V, Zuccato C, Gaudenzi G et al (2012) An evolutionary recent neuroepithelial cell adhesion function of huntingtin implicates ADAM10- Ncadherin. Nat Neurosci 15:713\u2013721. doi: 10.1038\/nn.3080","journal-title":"Nat Neurosci"},{"key":"477_CR66","doi-asserted-by":"publisher","first-page":"2433","DOI":"10.1038\/emboj.2010.117","volume":"29","author":"JD Godin","year":"2010","unstructured":"Godin JD, Poizat G, Hickey MA et al (2010) Mutant huntingtin-impaired degradation of \u03b2-catenin causes neurotoxicity in Huntington\u2019s disease. EMBO J 29:2433\u20132445. doi: 10.1038\/emboj.2010.117","journal-title":"EMBO J"},{"key":"477_CR67","doi-asserted-by":"publisher","first-page":"852","DOI":"10.1016\/j.biocel.2011.03.009","volume":"43","author":"JD Godin","year":"2011","unstructured":"Godin JD, Humbert S (2011) Mitotic spindle: focus on the function of huntingtin. Int J Biochem Cell Biol 43:852\u2013856. doi: 10.1016\/j.biocel.2011.03.009","journal-title":"Int J Biochem Cell Biol"},{"key":"477_CR68","doi-asserted-by":"publisher","first-page":"10034","DOI":"10.1523\/JNEUROSCI.0715-14.2014","volume":"34","author":"M Molina-Calavita","year":"2014","unstructured":"Molina-Calavita M, Barnat M, Elias S et al (2014) Mutant huntingtin affects cortical progenitor cell division and development of the mouse neocortex. J Neurosci Off J Soc Neurosci 34:10034\u201310040. doi: 10.1523\/JNEUROSCI.0715-14.2014","journal-title":"J Neurosci Off J Soc Neurosci"},{"key":"477_CR69","doi-asserted-by":"publisher","first-page":"9455","DOI":"10.1523\/JNEUROSCI.4699-13.2014","volume":"34","author":"SU McKinstry","year":"2014","unstructured":"McKinstry SU, Karadeniz YB, Worthington AK et al (2014) Huntingtin is required for normal excitatory synapse development in cortical and striatal circuits. J Neurosci Off J Soc Neurosci 34:9455\u20139472. doi: 10.1523\/JNEUROSCI.4699-13.2014","journal-title":"J Neurosci Off J Soc Neurosci"},{"key":"477_CR70","doi-asserted-by":"publisher","first-page":"21900","DOI":"10.1073\/pnas.0912171106","volume":"106","author":"AE Molero","year":"2009","unstructured":"Molero AE, Gokhan S, Gonzalez S et al (2009) Impairment of developmental stem cell-mediated striatal neurogenesis and pluripotency genes in a knock-in model of Huntington\u2019s disease. Proc Natl Acad Sci U S A 106:21900\u201321905. doi: 10.1073\/pnas.0912171106","journal-title":"Proc Natl Acad Sci U S A"},{"key":"477_CR71","doi-asserted-by":"publisher","first-page":"777","DOI":"10.1016\/j.neuroscience.2004.12.051","volume":"132","author":"MA Curtis","year":"2005","unstructured":"Curtis MA, Penney EB, Pearson J et al (2005) The distribution of progenitor cells in the subependymal layer of the lateral ventricle in the normal and Huntington\u2019s disease human brain. Neuroscience 132:777\u2013788. doi: 10.1016\/j.neuroscience.2004.12.051","journal-title":"Neuroscience"},{"key":"477_CR72","doi-asserted-by":"publisher","first-page":"663","DOI":"10.1016\/j.cell.2006.07.024","volume":"126","author":"K Takahashi","year":"2006","unstructured":"Takahashi K, Yamanaka S (2006) Induction of pluripotent stem cells from mouse embryonic and adult fibroblast cultures by defined factors. Cell 126:663\u2013676. doi: 10.1016\/j.cell.2006.07.024","journal-title":"Cell"},{"key":"477_CR73","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1186\/1471-2121-11-12","volume":"11","author":"C Laowtammathron","year":"2010","unstructured":"Laowtammathron C, Cheng EC, Cheng P-H et al (2010) Monkey hybrid stem cells develop cellular features of Huntington\u2019s disease. BMC Cell Biol 11:12. doi: 10.1186\/1471-2121-11-12","journal-title":"BMC Cell Biol"},{"key":"477_CR74","doi-asserted-by":"publisher","first-page":"1198","DOI":"10.1089\/scd.2012.0469","volume":"22","author":"K Putkhao","year":"2013","unstructured":"Putkhao K, Kocerha J, Cho I-K et al (2013) Pathogenic cellular phenotypes are germline transmissible in a transgenic primate model of Huntington\u2019s disease. Stem Cells Dev 22:1198\u20131205. doi: 10.1089\/scd.2012.0469","journal-title":"Stem Cells Dev"},{"key":"477_CR75","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/S1472-6483(10)60810-3","volume":"10","author":"Y Verlinsky","year":"2005","unstructured":"Verlinsky Y, Strelchenko N, Kukharenko V et al (2005) Human embryonic stem cell lines with genetic disorders. Reprod Biomed Online 10:105\u2013110","journal-title":"Reprod Biomed Online"},{"key":"477_CR76","doi-asserted-by":"publisher","first-page":"3883","DOI":"10.1093\/hmg\/dds216","volume":"21","author":"M Feyeux","year":"2012","unstructured":"Feyeux M, Bourgois-Rocha F, Redfern A et al (2012) Early transcriptional changes linked to naturally occurring Huntington\u2019s disease mutations in neural derivatives of human embryonic stem cells. Hum Mol Genet 21:3883\u20133895. doi: 10.1093\/hmg\/dds216","journal-title":"Hum Mol Genet"},{"key":"477_CR77","doi-asserted-by":"publisher","first-page":"503","DOI":"10.1093\/humrep\/dei345","volume":"21","author":"I Mateizel","year":"2006","unstructured":"Mateizel I, De Temmerman N, Ullmann U et al (2006) Derivation of human embryonic stem cell lines from embryos obtained after IVF and after PGD for monogenic disorders. Hum Reprod Oxf Engl 21:503\u2013511. doi: 10.1093\/humrep\/dei345","journal-title":"Hum Reprod Oxf Engl"},{"key":"477_CR78","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0099341","volume":"9","author":"V Drouet","year":"2014","unstructured":"Drouet V, Ruiz M, Zala D et al (2014) Allele-specific silencing of mutant huntingtin in rodent brain and human stem cells. PloS One 9:e99341. doi: 10.1371\/journal.pone.0099341","journal-title":"PloS One"},{"key":"477_CR79","doi-asserted-by":"publisher","first-page":"376","DOI":"10.1007\/s11626-010-9300-8","volume":"46","author":"P Tropel","year":"2010","unstructured":"Tropel P, Tournois J, C\u00f4me J et al (2010) High-efficiency derivation of human embryonic stem cell lines following pre-implantation genetic diagnosis. In Vitro Cell Dev Biol Anim 46:376\u2013385. doi: 10.1007\/s11626-010-9300-8","journal-title":"In Vitro Cell Dev Biol Anim"},{"key":"477_CR80","doi-asserted-by":"publisher","unstructured":"Bradley CK, Scott HA, Chami O, et al (2011) Derivation of Huntington\u2019s disease-affected human embryonic stem cell lines. Stem Cells Dev 20:495\u2013 502. doi: 10.1089\/scd.2010.0120","DOI":"10.1089\/scd.2010.0120"},{"key":"477_CR81","doi-asserted-by":"publisher","first-page":"e0126860","DOI":"10.1371\/journal.pone.0126860","volume":"10","author":"L Jacquet","year":"2015","unstructured":"Jacquet L, Neueder A, F\u00f6ldes G et al (2015) Three Huntington\u2019s Disease Specific Mutation-Carrying Human Embryonic Stem Cell Lines Have Stable Number of CAG Repeats upon In Vitro Differentiation into Cardiomyocytes. PloS One 10:e0126860. doi: 10.1371\/journal.pone.0126860","journal-title":"PloS One"},{"key":"477_CR82","doi-asserted-by":"publisher","first-page":"509","DOI":"10.1089\/cell.2010.0019","volume":"12","author":"AWS Chan","year":"2010","unstructured":"Chan AWS, Cheng P-H, Neumann A, Yang J-J (2010) Reprogramming Huntington monkey skin cells into pluripotent stem cells. Cell Reprogramming 12:509\u2013517. doi: 10.1089\/cell.2010.0019","journal-title":"Cell Reprogramming"},{"key":"477_CR83","doi-asserted-by":"publisher","first-page":"877","DOI":"10.1016\/j.cell.2008.07.041","volume":"134","author":"I-H Park","year":"2008","unstructured":"Park I-H, Arora N, Huo H et al (2008) Disease-specific induced pluripotent stem cells. Cell 134:877\u2013886. doi: 10.1016\/j.cell.2008.07.041","journal-title":"Cell"},{"key":"477_CR84","doi-asserted-by":"publisher","first-page":"306","DOI":"10.1016\/j.ajhg.2013.05.025","volume":"93","author":"P-H Cheng","year":"2013","unstructured":"Cheng P-H, Li C-L, Chang Y-F et al (2013) miR-196a ameliorates phenotypes of Huntington disease in cell, transgenic mouse, and induced pluripotent stem cell models. Am J Hum Genet 93:306\u2013312. doi: 10.1016\/j.ajhg.2013.05.025","journal-title":"Am J Hum Genet"},{"key":"477_CR85","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1186\/1756-6606-5-17","volume":"5","author":"TA Juopperi","year":"2012","unstructured":"Juopperi TA, Kim WR, Chiang C-H et al (2012) Astrocytes generated from patient induced pluripotent stem cells recapitulate features of Huntington\u2019s disease patient cells. Mol Brain 5:17. doi: 10.1186\/1756-6606-5-17","journal-title":"Mol Brain"},{"key":"477_CR86","doi-asserted-by":"publisher","first-page":"1929","DOI":"10.1093\/hmg\/ddu609","volume":"24","author":"AM Tidball","year":"2015","unstructured":"Tidball AM, Bryan MR, Uhouse MA et al (2015) A novel manganesedependent ATM-p53 signaling pathway is selectively impaired in patient-based neuroprogenitor and murine striatal models of Huntington\u2019s disease. Hum Mol Genet 24:1929\u20131944. doi: 10.1093\/hmg\/ddu609","journal-title":"Hum Mol Genet"},{"key":"477_CR87","doi-asserted-by":"publisher","first-page":"e0150372","DOI":"10.1371\/journal.pone.0150372","volume":"11","author":"AM Tidball","year":"2016","unstructured":"Tidball AM, Neely MD, Chamberlin R et al (2016) Genomic Instability Associated with p53 Knockdown in the Generation of Huntington\u2019s Disease Human Induced Pluripotent Stem Cells. PloS One 11:e0150372. doi: 10.1371\/journal.pone.0150372","journal-title":"PloS One"},{"key":"477_CR88","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1016\/j.ymeth.2015.11.017","volume":"98","author":"MO Urbanek","year":"2016","unstructured":"Urbanek MO, Krzyzosiak WJ (2016) RNA FISH for detecting expanded repeats in human diseases. Methods San Diego Calif 98:115\u2013123. doi: 10.1016\/j.ymeth.2015.11.017","journal-title":"Methods San Diego Calif"},{"key":"477_CR89","doi-asserted-by":"publisher","first-page":"1513","DOI":"10.1016\/j.bbadis.2016.05.015","volume":"1862","author":"MO Urbanek","year":"2016","unstructured":"Urbanek MO, Jazurek M, Switonski PM et al (2016) Nuclear speckles are detention centers for transcripts containing expanded CAG repeats. Biochim Biophys Acta 1862:1513\u20131520. doi: 10.1016\/j.bbadis.2016.05.015","journal-title":"Biochim Biophys Acta"},{"key":"477_CR90","doi-asserted-by":"publisher","first-page":"140","DOI":"10.1111\/cpr.12162","volume":"48","author":"IK Rony","year":"2015","unstructured":"Rony IK, Baten A, Bloomfield JA et al (2015) Inducing pluripotency in vitro: recent advances and highlights in induced pluripotent stem cells generation and pluripotency reprogramming. Cell Prolif 48:140\u2013156. doi: 10.1111\/cpr.12162","journal-title":"Cell Prolif"},{"key":"477_CR91","doi-asserted-by":"publisher","first-page":"516","DOI":"10.1016\/j.cell.2015.07.003","volume":"162","author":"Genetic Modifiers of Huntington\u2019s Disease (GeM-HD) Consortium","year":"2015","unstructured":"Genetic Modifiers of Huntington\u2019s Disease (GeM-HD) Consortium (2015) Identification of Genetic Factors that Modify Clinical Onset of Huntington\u2019s Disease. Cell 162:516\u2013526. doi: 10.1016\/j.cell.2015.07.003","journal-title":"Cell"},{"key":"477_CR92","doi-asserted-by":"publisher","first-page":"287","DOI":"10.1016\/j.ajhg.2015.12.018","volume":"98","author":"JW Keum","year":"2016","unstructured":"Keum JW, Shin A, Gillis T et al (2016) The HTT CAG-Expansion Mutation Determines Age at Death but Not Disease Duration in Huntington Disease. Am J Hum Genet 98:287\u2013298. doi: 10.1016\/j.ajhg.2015.12.018","journal-title":"Am J Hum Genet"},{"key":"477_CR93","doi-asserted-by":"publisher","first-page":"365","DOI":"10.1242\/dev.142679","volume":"144","author":"A Smith","year":"2017","unstructured":"Smith A (2017) Formative pluripotency: the executive phase in a developmental continuum. Dev Camb Engl 144:365\u2013373. doi: 10.1242\/dev.142679","journal-title":"Dev Camb Engl"},{"key":"477_CR94","doi-asserted-by":"crossref","unstructured":"Teslaa T, Teitell MA (2015) Pluripotent stem cell energy metabolism: an update. EMBO J 34:138\u2013153. doi: 10.15252\/embj.201490446","DOI":"10.15252\/embj.201490446"},{"key":"477_CR95","doi-asserted-by":"publisher","first-page":"177","DOI":"10.1038\/nature13551","volume":"511","author":"H Ma","year":"2014","unstructured":"Ma H, Morey R, O\u2019Neil RC et al (2014) Abnormalities in human pluripotent cells due to reprogramming mechanisms. Nature 511:177\u2013183. doi: 10.1038\/nature13551","journal-title":"Nature"},{"key":"477_CR96","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1016\/j.stem.2012.06.012","volume":"11","author":"A Wutz","year":"2012","unstructured":"Wutz A (2012) Epigenetic alterations in human pluripotent stem cells: a tale of two cultures. Cell Stem Cell 11:9\u201315. doi: 10.1016\/j.stem.2012.06.012","journal-title":"Cell Stem Cell"},{"key":"477_CR97","doi-asserted-by":"publisher","first-page":"149","DOI":"10.1016\/j.stem.2013.07.001","volume":"13","author":"G Liang","year":"2013","unstructured":"Liang G, Zhang Y (2013) Genetic and epigenetic variations in iPSCs: potential causes and implications for application. Cell Stem Cell 13:149\u2013159. doi: 10.1016\/j.stem.2013.07.001","journal-title":"Cell Stem Cell"},{"key":"477_CR98","doi-asserted-by":"crossref","first-page":"2088","DOI":"10.1523\/JNEUROSCI.17-06-02088.1997","volume":"17","author":"L Cai","year":"1997","unstructured":"Cai L, Hayes NL, Nowakowski RS (1997) Synchrony of clonal cell proliferation and contiguity of clonally related cells: production of mosaicism in the ventricular zone of developing mouse neocortex. J Neurosci Off J Soc Neurosci 17:2088\u20132100","journal-title":"J Neurosci Off J Soc Neurosci"},{"key":"477_CR99","doi-asserted-by":"publisher","first-page":"2831","DOI":"10.1002\/cne.23608","volume":"522","author":"M Karus","year":"2014","unstructured":"Karus M, Blaess S, Br\u00fcstle O (2014) Self-organization of neural tissue architectures from pluripotent stem cells. J Comp Neurol 522:2831\u20132844. doi: 10.1002\/cne.23608","journal-title":"J Comp Neurol"},{"key":"477_CR100","doi-asserted-by":"publisher","first-page":"176","DOI":"10.1038\/nrn2761","volume":"11","author":"L Conti","year":"2010","unstructured":"Conti L, Cattaneo E (2010) Neural stem cell systems: physiological players or in vitro entities? Nat Rev Neurosci 11:176\u2013187. doi: 10.1038\/nrn2761","journal-title":"Nat Rev Neurosci"},{"key":"477_CR101","doi-asserted-by":"crossref","first-page":"1009","DOI":"10.1046\/j.1471-4159.1999.0721009.x","volume":"72","author":"M Metzler","year":"1999","unstructured":"Metzler M, Chen N, Helgason CD et al (1999) Life without huntingtin: normal differentiation into functional neurons. J Neurochem 72:1009\u20131018","journal-title":"J Neurochem"},{"key":"477_CR102","doi-asserted-by":"publisher","first-page":"e283","DOI":"10.1371\/journal.pbio.0030283","volume":"3","author":"L Conti","year":"2005","unstructured":"Conti L, Pollard SM, Gorba T et al (2005) Niche-independent symmetrical selfrenewal of a mammalian tissue stem cell. PLoS Biol 3:e283. doi: 10.1371\/journal.pbio.0030283","journal-title":"PLoS Biol"},{"key":"477_CR103","doi-asserted-by":"publisher","first-page":"1434","DOI":"10.1002\/mds.26007","volume":"29","author":"EJ Wild","year":"2014","unstructured":"Wild EJ, Tabrizi SJ (2014) Targets for future clinical trials in Huntington\u2019s disease: What\u2019s in the pipeline? Mov Disord 29:1434\u20131445. doi: 10.1002\/mds.26007","journal-title":"Mov Disord"},{"key":"477_CR104","doi-asserted-by":"publisher","first-page":"578","DOI":"10.1002\/dev.20546","volume":"54","author":"KG Bath","year":"2012","unstructured":"Bath KG, Akins MR, Lee FS (2012) BDNF control of adult SVZ neurogenesis. Dev Psychobiol 54:578\u2013589. doi: 10.1002\/dev.20546","journal-title":"Dev Psychobiol"},{"key":"477_CR105","doi-asserted-by":"publisher","first-page":"320","DOI":"10.1016\/j.nbd.2009.02.007","volume":"34","author":"D Spiliotopoulos","year":"2009","unstructured":"Spiliotopoulos D, Goffredo D, Conti L et al (2009) An optimized experimental strategy for efficient conversion of embryonic stem (ES)-derived mouse neural stem (NS) cells into a nearly homogeneous mature neuronal population. Neurobiol Dis 34:320\u2013331. doi: 10.1016\/j.nbd.2009.02.007","journal-title":"Neurobiol Dis"},{"key":"477_CR106","doi-asserted-by":"publisher","first-page":"142","DOI":"10.3109\/10409238.2014.999192","volume":"50","author":"K Usdin","year":"2015","unstructured":"Usdin K, House NCM, Freudenreich CH (2015) Repeat instability during DNA repair: Insights from model systems. Crit Rev Biochem Mol Biol 50:142\u2013167. doi: 10.3109\/10409238.2014.999192","journal-title":"Crit Rev Biochem Mol Biol"},{"key":"477_CR107","doi-asserted-by":"publisher","first-page":"786","DOI":"10.1038\/nrg2828","volume":"11","author":"CT McMurray","year":"2010","unstructured":"McMurray CT (2010) Mechanisms of trinucleotide repeat instability during human development. Nat Rev Genet 11:786\u2013799. doi: 10.1038\/nrg2828","journal-title":"Nat Rev Genet"},{"key":"477_CR108","doi-asserted-by":"publisher","first-page":"1133","DOI":"10.1093\/hmg\/ddm054","volume":"16","author":"PF Shelbourne","year":"2007","unstructured":"Shelbourne PF, Keller-McGandy C, Bi WL et al (2007) Triplet repeat mutation length gains correlate with cell-type specific vulnerability in Huntington disease brain. Hum Mol Genet 16:1133\u20131142. doi: 10.1093\/hmg\/ddm054","journal-title":"Hum Mol Genet"},{"key":"477_CR109","doi-asserted-by":"publisher","first-page":"e1005267","DOI":"10.1371\/journal.pgen.1005267","volume":"11","author":"H Budworth","year":"2015","unstructured":"Budworth H, Harris FR, Williams P et al (2015) Suppression of Somatic Expansion Delays the Onset of Pathophysiology in a Mouse Model of Huntington\u2019s Disease. PLoS Genet 11:e1005267. doi: 10.1371\/journal.pgen.1005267","journal-title":"PLoS Genet"},{"key":"477_CR110","doi-asserted-by":"publisher","first-page":"20948","DOI":"10.3390\/ijms151120948","volume":"15","author":"SP Wyles","year":"2014","unstructured":"Wyles SP, Brandt EB, Nelson TJ (2014) Stem cells: the pursuit of genomic stability. Int J Mol Sci 15:20948\u201320967. doi: 10.3390\/ijms151120948","journal-title":"Int J Mol Sci"},{"key":"477_CR111","doi-asserted-by":"publisher","first-page":"153","DOI":"10.1083\/jcb.201310135","volume":"204","author":"U Weissbein","year":"2014","unstructured":"Weissbein U, Benvenisty N, Ben-David U (2014) Quality control: Genome maintenance in pluripotent stem cells. J Cell Biol 204:153\u2013163. doi: 10.1083\/jcb.201310135","journal-title":"J Cell Biol"},{"key":"477_CR112","doi-asserted-by":"publisher","first-page":"8036","DOI":"10.1038\/ncomms9036","volume":"6","author":"S Ruiz","year":"2015","unstructured":"Ruiz S, Lopez-Contreras AJ, Gabut M et al (2015) Limiting replication stress during somatic cell reprogramming reduces genomic instability in induced pluripotent stem cells. Nat Commun 6:8036. doi: 10.1038\/ncomms9036","journal-title":"Nat Commun"},{"key":"477_CR113","doi-asserted-by":"publisher","first-page":"395","DOI":"10.3233\/JHD-130082","volume":"2","author":"C Marques Sousa","year":"2013","unstructured":"Marques Sousa C, Humbert S (2013) Huntingtin: here, there, everywhere! J Huntingt Dis 2:395\u2013403. doi: 10.3233\/JHD-130082","journal-title":"J Huntingt Dis"},{"key":"477_CR114","doi-asserted-by":"crossref","first-page":"5523","DOI":"10.1523\/JNEUROSCI.16-17-05523.1996","volume":"16","author":"PG Bhide","year":"1996","unstructured":"Bhide PG, Day M, Sapp E et al (1996) Expression of normal and mutant huntingtin in the developing brain. J Neurosci Off J Soc Neurosci 16:5523\u20135535","journal-title":"J Neurosci Off J Soc Neurosci"},{"key":"477_CR115","doi-asserted-by":"publisher","first-page":"e0127687","DOI":"10.1371\/journal.pone.0127687","volume":"10","author":"A Ruzo","year":"2015","unstructured":"Ruzo A, Ismailoglu I, Popowski M et al (2015) Discovery of novel isoforms of huntingtin reveals a new hominid-specific exon. PloS One 10:e0127687. doi: 10.1371\/journal.pone.0127687","journal-title":"PloS One"},{"key":"477_CR116","doi-asserted-by":"publisher","first-page":"1428","DOI":"10.1016\/j.jmb.2013.12.028","volume":"426","author":"AC Hughes","year":"2014","unstructured":"Hughes AC, Mort M, Elliston L et al (2014) Identification of novel alternative splicing events in the huntingtin gene and assessment of the functional consequences using structural protein homology modelling. J Mol Biol 426:1428\u20131438. doi: 10.1016\/j.jmb.2013.12.028","journal-title":"J Mol Biol"},{"key":"477_CR117","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1093\/hmg\/3.1.85","volume":"3","author":"B Lin","year":"1994","unstructured":"Lin B, Nasir J, MacDonald H et al (1994) Sequence of the murine Huntington disease gene: evidence for conservation, alternate splicing and polymorphism in a triplet (CCG) repeat [corrected]. Hum Mol Genet 3:85\u201392","journal-title":"Hum Mol Genet"},{"key":"477_CR118","doi-asserted-by":"publisher","first-page":"2366","DOI":"10.1073\/pnas.1221891110","volume":"110","author":"K Sathasivam","year":"2013","unstructured":"Sathasivam K, Neueder A, Gipson TA et al (2013) Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease. Proc Natl Acad Sci U S A 110:2366\u20132370. doi: 10.1073\/pnas.1221891110","journal-title":"Proc Natl Acad Sci U S A"},{"key":"477_CR119","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.expneurol.2011.12.013","volume":"238","author":"M Arrasate","year":"2012","unstructured":"Arrasate M, Finkbeiner S (2012) Protein aggregates in Huntington\u2019s disease. Exp Neurol 238:1\u201311. doi: 10.1016\/j.expneurol.2011.12.013","journal-title":"Exp Neurol"},{"key":"477_CR120","doi-asserted-by":"publisher","first-page":"91","DOI":"10.3390\/brainsci4010091","volume":"4","author":"G Hoffner","year":"2014","unstructured":"Hoffner G, Djian P (2014) Monomeric, oligomeric and polymeric proteins in huntington disease and other diseases of polyglutamine expansion. Brain Sci 4:91\u2013122. doi: 10.3390\/brainsci4010091","journal-title":"Brain Sci"},{"key":"477_CR121","doi-asserted-by":"publisher","first-page":"D793","DOI":"10.1093\/nar\/gks1055","volume":"41","author":"A Kamburov","year":"2013","unstructured":"Kamburov A, Stelzl U, Lehrach H, Herwig R (2013) The ConsensusPathDB interaction database: 2013 update. Nucleic Acids Res 41:D793\u2013D800. doi: 10.1093\/nar\/gks1055","journal-title":"Nucleic Acids Res"},{"key":"477_CR122","unstructured":"R Core Team (2016) R: A language and environment for statistical computing. R Foundation for Statistical Computing"},{"key":"477_CR123","unstructured":"Warnes G, Bolker B, Bonebakker L Gplots: Various R Programming Tools for Plotting Data. R package version 3.0.1"},{"key":"477_CR124","doi-asserted-by":"publisher","first-page":"541259","DOI":"10.1155\/2013\/541259","volume":"2013","author":"G Ellrichmann","year":"2013","unstructured":"Ellrichmann G, Reick C, Saft C, Linker RA (2013) The role of the immune system in Huntington\u2019s disease. Clin Dev Immunol 2013:541259. doi: 10.1155\/2013\/541259","journal-title":"Clin Dev Immunol"},{"key":"477_CR125","doi-asserted-by":"publisher","first-page":"89","DOI":"10.3233\/JHD-140106","volume":"3","author":"KR Bowles","year":"2014","unstructured":"Bowles KR, Jones L (2014) Kinase signalling in Huntington\u2019s disease. J Huntingt Dis 3:89\u2013123. doi: 10.3233\/JHD-140106","journal-title":"J Huntingt Dis"},{"key":"477_CR126","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a008151","author":"MA Basson","year":"2012","unstructured":"Basson MA (2012) Signaling in cell differentiation and morphogenesis. Cold Spring Harb Perspect Biol. doi: 10.1101\/cshperspect.a008151","journal-title":"Cold Spring Harb Perspect Biol."},{"key":"477_CR127","doi-asserted-by":"publisher","first-page":"1501","DOI":"10.1093\/carcin\/bgq101","volume":"31","author":"A Molchadsky","year":"2010","unstructured":"Molchadsky A, Rivlin N, Brosh R et al (2010) p53 is balancing development, differentiation and de-differentiation to assure cancer prevention. Carcinogenesis 31:1501\u20131508. doi: 10.1093\/carcin\/bgq101","journal-title":"Carcinogenesis"},{"key":"477_CR128","doi-asserted-by":"publisher","first-page":"576","DOI":"10.1038\/embor.2009.89","volume":"10","author":"A Tedeschi","year":"2009","unstructured":"Tedeschi A, Di Giovanni S (2009) The non-apoptotic role of p53 in neuronal biology: enlightening the dark side of the moon. EMBO Rep 10:576\u2013583. doi: 10.1038\/embor.2009.89","journal-title":"EMBO Rep"},{"key":"477_CR129","doi-asserted-by":"publisher","first-page":"927","DOI":"10.1038\/sj.cdd.4401912","volume":"13","author":"J-C Marine","year":"2006","unstructured":"Marine J-C, Francoz S, Maetens M et al (2006) Keeping p53 in check: essential and synergistic functions of Mdm2 and Mdm4. Cell Death Differ 13:927\u2013934. doi: 10.1038\/sj.cdd.4401912","journal-title":"Cell Death Differ"},{"key":"477_CR130","doi-asserted-by":"publisher","first-page":"228","DOI":"10.1038\/nature13585","volume":"514","author":"JL Van Nostrand","year":"2014","unstructured":"Van Nostrand JL, Brady CA, Jung H et al (2014) Inappropriate p53 activation during development induces features of CHARGE syndrome. Nature 514:228\u2013232. doi: 10.1038\/nature13585","journal-title":"Nature"},{"key":"477_CR131","doi-asserted-by":"publisher","first-page":"419","DOI":"10.1016\/j.nbd.2006.08.002","volume":"24","author":"AB Ryan","year":"2006","unstructured":"Ryan AB, Zeitlin SO, Scrable H (2006) Genetic interaction between expanded murine Hdh alleles and p53 reveal deleterious effects of p53 on Huntington\u2019s disease pathogenesis. Neurobiol Dis 24:419\u2013427. doi: 10.1016\/j.nbd.2006.08.002","journal-title":"Neurobiol Dis"},{"key":"477_CR132","doi-asserted-by":"publisher","first-page":"2944","DOI":"10.1007\/s12035-015-9188-0","volume":"53","author":"N Brustovetsky","year":"2016","unstructured":"Brustovetsky N (2016) Mutant Huntingtin and Elusive Defects in Oxidative Metabolism and Mitochondrial Calcium Handling. Mol Neurobiol 53:2944\u20132953. doi: 10.1007\/s12035-015-9188-0","journal-title":"Mol Neurobiol"},{"key":"477_CR133","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1016\/j.nbd.2015.09.008","volume":"90","author":"P Guedes-Dias","year":"2016","unstructured":"Guedes-Dias P, Pinho BR, Soares TR et al (2016) Mitochondrial dynamics and quality control in Huntington\u2019s disease. Neurobiol Dis 90:51\u201357. doi: 10.1016\/j.nbd.2015.09.008","journal-title":"Neurobiol Dis"},{"key":"477_CR134","doi-asserted-by":"publisher","first-page":"493","DOI":"10.1172\/JCI45691","volume":"121","author":"F Mochel","year":"2011","unstructured":"Mochel F, Haller RG (2011) Energy deficit in Huntington disease: why it matters. J Clin Invest 121:493\u2013499. doi: 10.1172\/JCI45691","journal-title":"J Clin Invest"},{"key":"477_CR135","doi-asserted-by":"crossref","first-page":"5574","DOI":"10.2174\/1381612820666140305224906","volume":"20","author":"M Uittenbogaard","year":"2014","unstructured":"Uittenbogaard M, Chiaramello A (2014) Mitochondrial biogenesis: a therapeutic target for neurodevelopmental disorders and neurodegenerative diseases. Curr Pharm Des 20:5574\u20135593","journal-title":"Curr Pharm Des"},{"key":"477_CR136","doi-asserted-by":"publisher","first-page":"519","DOI":"10.1038\/nature06968","volume":"453","author":"Q-L Ying","year":"2008","unstructured":"Ying Q-L, Wray J, Nichols J et al (2008) The ground state of embryonic stem cell self-renewal. Nature 453:519\u2013523. doi: 10.1038\/nature06968","journal-title":"Nature"},{"key":"477_CR137","doi-asserted-by":"publisher","first-page":"1095","DOI":"10.1016\/j.bbalip.2014.12.018","volume":"1851","author":"V Leoni","year":"2015","unstructured":"Leoni V, Caccia C (2015) The impairment of cholesterol metabolism in Huntington disease. Biochim Biophys Acta 1851:1095\u20131105. doi: 10.1016\/j.bbalip.2014.12.018","journal-title":"Biochim Biophys Acta"},{"key":"477_CR138","doi-asserted-by":"publisher","first-page":"217","DOI":"10.3233\/JHD-160205","volume":"5","author":"A Kumar","year":"2016","unstructured":"Kumar A, Ratan RR (2016) Oxidative Stress and Huntington\u2019s Disease: The Good, The Bad, and The Ugly. J Huntingt Dis 5:217\u2013237. doi: 10.3233\/JHD-160205","journal-title":"J Huntingt Dis"},{"key":"477_CR139","doi-asserted-by":"publisher","first-page":"4206","DOI":"10.1242\/dev.107086","volume":"141","author":"CL Bigarella","year":"2014","unstructured":"Bigarella CL, Liang R, Ghaffari S (2014) Stem cells and the impact of ROS signaling. Dev Camb Engl 141:4206\u20134218. doi: 10.1242\/dev.107086","journal-title":"Dev Camb Engl"},{"key":"477_CR140","doi-asserted-by":"publisher","first-page":"964518","DOI":"10.1155\/2015\/964518","volume":"2015","author":"H Prentice","year":"2015","unstructured":"Prentice H, Modi JP, Wu J-Y (2015) Mechanisms of Neuronal Protection against Excitotoxicity, Endoplasmic Reticulum Stress, and Mitochondrial Dysfunction in Stroke and Neurodegenerative Diseases. Oxid Med Cell Longev 2015:964518. doi: 10.1155\/2015\/964518","journal-title":"Oxid Med Cell Longev"},{"key":"477_CR141","doi-asserted-by":"publisher","first-page":"905","DOI":"10.1152\/physrev.00041.2009","volume":"90","author":"C Zuccato","year":"2010","unstructured":"Zuccato C, Valenza M, Cattaneo E (2010) Molecular mechanisms and potential therapeutical targets in Huntington\u2019s disease. Physiol Rev 90:905\u2013981. doi: 10.1152\/physrev.00041.2009","journal-title":"Physiol Rev"},{"key":"477_CR142","doi-asserted-by":"publisher","first-page":"951","DOI":"10.4161\/auto.24273","volume":"9","author":"AN Hale","year":"2013","unstructured":"Hale AN, Ledbetter DJ, Gawriluk TR, Rucker EB (2013) Autophagy: regulation and role in development. Autophagy 9:951\u2013972. doi: 10.4161\/auto.24273","journal-title":"Autophagy"},{"key":"477_CR143","doi-asserted-by":"publisher","first-page":"830","DOI":"10.4161\/auto.24132","volume":"9","author":"J-L Guan","year":"2013","unstructured":"Guan J-L, Simon AK, Prescott M et al (2013) Autophagy in stem cells. Autophagy 9:830\u2013849. doi: 10.4161\/auto.24132","journal-title":"Autophagy"},{"key":"477_CR144","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1016\/j.tins.2014.09.003","volume":"38","author":"DDO Martin","year":"2015","unstructured":"Martin DDO, Ladha S, Ehrnhoefer DE, Hayden MR (2015) Autophagy in Huntington disease and huntingtin in autophagy. Trends Neurosci 38:26\u201335. doi: 10.1016\/j.tins.2014.09.003","journal-title":"Trends Neurosci"},{"key":"477_CR145","doi-asserted-by":"publisher","first-page":"963","DOI":"10.1016\/j.drudis.2014.02.014","volume":"19","author":"CJ Cortes","year":"2014","unstructured":"Cortes CJ, La Spada AR (2014) The many faces of autophagy dysfunction in Huntington\u2019s disease: from mechanism to therapy. Drug Discov Today 19:963\u2013971. doi: 10.1016\/j.drudis.2014.02.014","journal-title":"Drug Discov Today"},{"key":"477_CR146","doi-asserted-by":"publisher","first-page":"103","DOI":"10.12688\/f1000research.6358.2","volume":"4","author":"RKR Kalathur","year":"2015","unstructured":"Kalathur RKR, Giner-Lamia J, Machado S et al (2015) The unfolded protein response and its potential role in Huntington\u2019s disease elucidated by a systems biology approach. F1000Research 4:103. doi: 10.12688\/f1000research.6358.2","journal-title":"F1000Research"},{"key":"477_CR147","doi-asserted-by":"publisher","first-page":"424","DOI":"10.1038\/sj.cdd.4401358","volume":"11","author":"E Hermel","year":"2004","unstructured":"Hermel E, Gafni J, Propp SS et al (2004) Specific caspase interactions and amplification are involved in selective neuronal vulnerability in Huntington\u2019s disease. Cell Death Differ 11:424\u2013438. doi: 10.1038\/sj.cdd.4401358","journal-title":"Cell Death Differ"},{"key":"477_CR148","doi-asserted-by":"publisher","first-page":"14794","DOI":"10.1523\/JNEUROSCI.2774-11.2011","volume":"31","author":"Y Tong","year":"2011","unstructured":"Tong Y, Ha TJ, Liu L et al (2011) Spatial and temporal requirements for huntingtin (Htt) in neuronal migration and survival during brain development. J Neurosci Off J Soc Neurosci 31:14794\u201314799. doi: 10.1523\/JNEUROSCI.2774-11.2011","journal-title":"J Neurosci Off J Soc Neurosci"},{"key":"477_CR149","doi-asserted-by":"publisher","first-page":"1179","DOI":"10.1016\/j.cell.2006.04.026","volume":"125","author":"RK Graham","year":"2006","unstructured":"Graham RK, Deng Y, Slow EJ et al (2006) Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell 125:1179\u20131191. doi: 10.1016\/j.cell.2006.04.026","journal-title":"Cell"},{"key":"477_CR150","doi-asserted-by":"crossref","first-page":"3705","DOI":"10.1523\/JNEUROSCI.20-10-03705.2000","volume":"20","author":"D Rigamonti","year":"2000","unstructured":"Rigamonti D, Bauer JH, De-Fraja C et al (2000) Wild-type huntingtin protects from apoptosis upstream of caspase-3. J Neurosci Off J Soc Neurosci 20:3705\u20133713","journal-title":"J Neurosci Off J Soc Neurosci"},{"key":"477_CR151","doi-asserted-by":"publisher","first-page":"5896","DOI":"10.1038\/sj.emboj.7601445","volume":"25","author":"Y Zhang","year":"2006","unstructured":"Zhang Y, Leavitt BR, van Raamsdonk JM et al (2006) Huntingtin inhibits caspase-3 activation. EMBO J 25:5896\u20135906. doi: 10.1038\/sj.emboj.7601445","journal-title":"EMBO J"},{"key":"477_CR152","doi-asserted-by":"publisher","first-page":"183","DOI":"10.1385\/NMM:1:3:183","volume":"1","author":"T Kiechle","year":"2002","unstructured":"Kiechle T, Dedeoglu A, Kubilus J et al (2002) Cytochrome C and caspase-9 expression in Huntington\u2019s disease. Neuromolecular Med 1:183\u2013195. doi: 10.1385\/NMM:1:3:183","journal-title":"Neuromolecular Med"},{"key":"477_CR153","doi-asserted-by":"publisher","first-page":"1599","DOI":"10.1007\/s00702-011-0646-1","volume":"118","author":"F Squitieri","year":"1996","unstructured":"Squitieri F, Maglione V, Orobello S (1996) Fornai F (2011) Genotype-, agingdependent abnormal caspase activity in Huntington disease blood cells. J Neural Transm Vienna Austria 118:1599\u20131607. doi: 10.1007\/s00702-011-0646-1","journal-title":"J Neural Transm Vienna Austria"},{"key":"477_CR154","doi-asserted-by":"publisher","first-page":"597","DOI":"10.1152\/physrev.00014.2011","volume":"92","author":"S Hirano","year":"2012","unstructured":"Hirano S, Takeichi M (2012) Cadherins in brain morphogenesis and wiring. Physiol Rev 92:597\u2013634. doi: 10.1152\/physrev.00014.2011","journal-title":"Physiol Rev"},{"key":"477_CR155","doi-asserted-by":"publisher","first-page":"394","DOI":"10.3389\/fncel.2015.00394","volume":"9","author":"T Kawauchi","year":"2015","unstructured":"Kawauchi T (2015) Cellullar insights into cerebral cortical development: focusing on the locomotion mode of neuronal migration. Front Cell Neurosci 9:394. doi: 10.3389\/fncel.2015.00394","journal-title":"Front Cell Neurosci"},{"key":"477_CR156","doi-asserted-by":"publisher","first-page":"2344","DOI":"10.1093\/hmg\/ddr127","volume":"20","author":"SA Reis","year":"2011","unstructured":"Reis SA, Thompson MN, Lee J-M et al (2011) Striatal neurons expressing fulllength mutant huntingtin exhibit decreased N-cadherin and altered neuritogenesis. Hum Mol Genet 20:2344\u20132355. doi: 10.1093\/hmg\/ddr127","journal-title":"Hum Mol Genet"},{"key":"477_CR157","doi-asserted-by":"publisher","first-page":"591","DOI":"10.1016\/j.stem.2015.05.004","volume":"16","author":"L Studer","year":"2015","unstructured":"Studer L, Vera E, Cornacchia D (2015) Programming and Reprogramming Cellular Age in the Era of Induced Pluripotency. Cell Stem Cell 16:591\u2013600. doi: 10.1016\/j.stem.2015.05.004","journal-title":"Cell Stem Cell"},{"key":"477_CR158","doi-asserted-by":"publisher","unstructured":"Tousley A, Kegel-Gleason KB Induced Pluripotent Stem Cells in Huntington\u2019s Disease Research: Progress and Opportunity. J Huntingt Dis 5:99\u2013131. doi: 10.3233\/JHD-160199","DOI":"10.3233\/JHD-160199"},{"key":"477_CR159","doi-asserted-by":"publisher","first-page":"585","DOI":"10.1212\/WNL.0000000000002939","volume":"87","author":"L Chang","year":"2016","unstructured":"Chang L, Douet V, Bloss C et al (2016) Gray matter maturation and cognition in children with different APOE \u03b5 genotypes. Neurology 87:585\u2013594. doi: 10.1212\/WNL.0000000000002939","journal-title":"Neurology"}],"container-title":["Molecular Neurobiology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s12035-017-0477-7\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s12035-017-0477-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s12035-017-0477-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,9,24]],"date-time":"2019-09-24T08:44:27Z","timestamp":1569314667000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s12035-017-0477-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,5,11]]},"references-count":159,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2018,4]]}},"alternative-id":["477"],"URL":"https:\/\/doi.org\/10.1007\/s12035-017-0477-7","relation":{},"ISSN":["0893-7648","1559-1182"],"issn-type":[{"value":"0893-7648","type":"print"},{"value":"1559-1182","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,5,11]]}}}