{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T11:50:17Z","timestamp":1790769017684,"version":"4.1.0"},"reference-count":59,"publisher":"Frontiers Media SA","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"}]},{"DOI":"10.13039\/501100004569","name":"Ministerstwo Nauki i Szkolnictwa Wy\u017cszego","doi-asserted-by":"publisher","award":["01\/KNOW2\/2014"],"award-info":[{"award-number":["01\/KNOW2\/2014"]}],"id":[{"id":"10.13039\/501100004569","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Mol. Neurosci."],"DOI":"10.3389\/fnmol.2017.00253","type":"journal-article","created":{"date-parts":[[2017,8,8]],"date-time":"2017-08-08T04:15:15Z","timestamp":1502165715000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":17,"title":["The Generation of Mouse and Human Huntington Disease iPS Cells Suitable for In vitro Studies on Huntingtin Function"],"prefix":"10.3389","volume":"10","author":[{"given":"Wojciech J.","family":"Szlachcic","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kalina","family":"Wiatr","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":"1965","published-online":{"date-parts":[[2017,8,8]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"253","DOI":"10.1016\/j.stem.2012.04.026","article-title":"Genetic correction of Huntington's disease phenotypes in induced pluripotent stem cells","volume":"11","author":"An","year":"2012","journal-title":"Cell Stem Cell"},{"key":"B2","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1016\/j.neuron.2005.06.005","article-title":"p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease","volume":"47","author":"Bae","year":"2005","journal-title":"Neuron"},{"key":"B3","doi-asserted-by":"publisher","first-page":"15005","DOI":"10.1038\/nrdp.2015.5","article-title":"Huntington disease","volume":"1","author":"Bates","year":"2015","journal-title":"Nat. Rev. Dis. Primers"},{"key":"B4","doi-asserted-by":"publisher","first-page":"89","DOI":"10.3233\/JHD-140106","article-title":"Kinase signalling in Huntington's disease","volume":"3","author":"Bowles","year":"2014","journal-title":"J. Huntingtons Dis."},{"key":"B5","doi-asserted-by":"publisher","first-page":"301","DOI":"10.1038\/nrm2873","article-title":"Prion-like transmission of protein aggregates in neurodegenerative diseases","volume":"11","author":"Brundin","year":"2010","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"B6","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1002\/ana.24174","article-title":"Mutant huntingtin is present in neuronal grafts in Huntington disease patients","volume":"76","author":"Cicchetti","year":"2014","journal-title":"Ann. Neurol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"12483","DOI":"10.1073\/pnas.0904239106","article-title":"Neural transplants in patients with Huntington's disease undergo disease-like neuronal degeneration","volume":"106","author":"Cicchetti","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"B8","doi-asserted-by":"publisher","first-page":"641","DOI":"10.1093\/brain\/awq328","article-title":"Neuronal degeneration in striatal transplants and Huntington's disease: potential mechanisms and clinical implications","volume":"134","author":"Cicchetti","year":"2011","journal-title":"Brain"},{"key":"B9","doi-asserted-by":"publisher","first-page":"3678","DOI":"10.1242\/jcs.126086","article-title":"Transfer of polyglutamine aggregates in neuronal cells occurs in tunneling nanotubes","volume":"126","author":"Costanzo","year":"2013","journal-title":"J. Cell. Sci."},{"key":"B10","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1126\/science.7618107","article-title":"Inactivation of the mouse Huntington's disease gene homolog Hdh","volume":"269","author":"Duyao","year":"1995","journal-title":"Science"},{"key":"B11","doi-asserted-by":"publisher","first-page":"417","DOI":"10.1016\/j.scr.2013.01.009","article-title":"EZ spheres: a stable and expandable culture system for the generation of pre-rosette multipotent stem cells from human ESCs and iPSCs","volume":"10","author":"Ebert","year":"2013","journal-title":"Stem Cell Res."},{"key":"B12","doi-asserted-by":"publisher","first-page":"717","DOI":"10.1093\/hmg\/ddt458","article-title":"p53 increases caspase-6 expression and activation in muscle tissue expressing mutant huntingtin","volume":"23","author":"Ehrnhoefer","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"B13","doi-asserted-by":"publisher","first-page":"10426","DOI":"10.1093\/nar\/gkt825","article-title":"Self-duplexing CUG repeats selectively inhibit mutant huntingtin expression","volume":"41","author":"Fiszer","year":"2013","journal-title":"Nucleic Acids Res."},{"key":"B14","doi-asserted-by":"publisher","first-page":"182","DOI":"10.3389\/fncel.2013.00182","article-title":"Extracellular vesicles as mediators of neuron-glia communication","volume":"7","author":"Fr\u00fchbeis","year":"2013","journal-title":"Front. Cell. Neurosci."},{"key":"B15","doi-asserted-by":"crossref","first-page":"6029","DOI":"10.1523\/JNEUROSCI.22-14-06029.2002","article-title":"A critical function of the pial basement membrane in cortical histogenesis","volume":"22","author":"Halfter","year":"2002","journal-title":"J. Neurosci."},{"key":"B16","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1016\/j.febslet.2011.11.019","article-title":"\u03b1-Synuclein modifies huntingtin aggregation in living cells","volume":"586","author":"Herrera","year":"2012","journal-title":"FEBS Lett."},{"key":"B17","doi-asserted-by":"crossref","first-page":"3310","DOI":"10.1523\/JNEUROSCI.05-12-03310.1985","article-title":"Identification of major cell classes in the developing mammalian nervous system","volume":"5","author":"Hockfield","year":"1985","journal-title":"J. Neurosci."},{"key":"B18","doi-asserted-by":"publisher","first-page":"56","DOI":"10.3389\/fgene.2012.00056","article-title":"Exosomal miRNAs: biological properties and therapeutic potential","volume":"3","author":"Hu","year":"2012","journal-title":"Front. Genet."},{"key":"B19","doi-asserted-by":"publisher","first-page":"577","DOI":"10.1007\/s00401-016-1582-9","article-title":"Human-to-mouse prion-like propagation of mutant huntingtin protein","volume":"132","author":"Jeon","year":"2016","journal-title":"Acta Neuropathol."},{"key":"B20","doi-asserted-by":"publisher","first-page":"319","DOI":"10.1016\/S0361-9230(01)00599-8","article-title":"New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins","volume":"56","author":"Ko","year":"2001","journal-title":"Brain Res. Bull."},{"key":"B21","doi-asserted-by":"publisher","first-page":"1031","DOI":"10.1016\/j.neuron.2012.05.009","article-title":"Sustained therapeutic reversal of Huntington's disease by transient repression of huntingtin synthesis","volume":"74","author":"Kordasiewicz","year":"2012","journal-title":"Neuron"},{"key":"B22","doi-asserted-by":"publisher","first-page":"336r","DOI":"10.1126\/scitranslmed.aad9370","article-title":"MDM2 inhibition rescues neurogenic and cognitive deficits in a mouse model of fragile X syndrome","volume":"8","author":"Li","year":"2016","journal-title":"Sci. Transl. Med."},{"key":"B23","doi-asserted-by":"publisher","first-page":"e96854","DOI":"10.1371\/journal.pone.0096854","article-title":"Quantification assays for total and polyglutamine-expanded huntingtin proteins","volume":"9","author":"Macdonald","year":"2014","journal-title":"PLoS ONE"},{"key":"B24","doi-asserted-by":"publisher","first-page":"779","DOI":"10.1111\/bpa.12427","article-title":"RNA toxicity induced by expanded CAG repeats in Huntington's disease","volume":"26","author":"Mart\u00ed","year":"2016","journal-title":"Brain Pathol."},{"key":"B25","doi-asserted-by":"publisher","first-page":"76","DOI":"10.1016\/j.brainres.2015.10.001","article-title":"Modeling Huntington's disease with patient-derived neurons","volume":"1656","author":"Mattis","year":"2015","journal-title":"Brain Res."},{"key":"B26","doi-asserted-by":"publisher","first-page":"9455","DOI":"10.1523\/JNEUROSCI.4699-13.2014","article-title":"Huntingtin is required for normal excitatory synapse development in cortical and striatal circuits","volume":"34","author":"McKinstry","year":"2014","journal-title":"J. Neurosci."},{"key":"B27","doi-asserted-by":"publisher","first-page":"1135","DOI":"10.1042\/BST20140142","article-title":"Regulation of miRNA strand selection: follow the leader?","volume":"42","author":"Meijer","year":"2014","journal-title":"Biochem. Soc. Trans."},{"key":"B28","doi-asserted-by":"publisher","first-page":"431","DOI":"10.1177\/1947601911409736","article-title":"p53 in the CNS: perspectives on development, stem cells, and cancer","volume":"2","author":"Mendrysa","year":"2011","journal-title":"Genes Cancer"},{"key":"B29","doi-asserted-by":"publisher","first-page":"e297","DOI":"10.1038\/mtna.2016.7","article-title":"Design, Characterization, and lead selection of therapeutic miRNAs targeting huntingtin for development of gene therapy for Huntington's disease","volume":"5","author":"Miniarikova","year":"2016","journal-title":"Mol. Ther. Nucleic Acids"},{"key":"B30","doi-asserted-by":"publisher","first-page":"5736","DOI":"10.1073\/pnas.1603871113","article-title":"Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington's disease","volume":"113","author":"Molero","year":"2016","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"B31","doi-asserted-by":"publisher","first-page":"21900","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","volume":"106","author":"Molero","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"B32","doi-asserted-by":"publisher","first-page":"10034","DOI":"10.1523\/JNEUROSCI.0715-14.2014","article-title":"Mutant huntingtin affects cortical progenitor cell division and development of the mouse neocortex","volume":"34","author":"Molina-Calavita","year":"2014","journal-title":"J. Neurosci."},{"key":"B33","doi-asserted-by":"publisher","first-page":"811","DOI":"10.1016\/0092-8674(95)90542-1","article-title":"Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes","volume":"81","author":"Nasir","year":"1995","journal-title":"Cell"},{"key":"B34","doi-asserted-by":"publisher","first-page":"314","DOI":"10.1177\/1073858416667270","article-title":"How Wnt signaling builds the brain: bridging development and disease","volume":"23","author":"Noelanders","year":"2017","journal-title":"Neuroscientist"},{"key":"B35","doi-asserted-by":"publisher","first-page":"D153","DOI":"10.1093\/nar\/gkj051","article-title":"RNAi Codex: a portal\/database for short-hairpin RNA (shRNA) gene-silencing constructs","volume":"34","author":"Olson","year":"2006","journal-title":"Nucleic Acids Res."},{"key":"B36","doi-asserted-by":"publisher","first-page":"271","DOI":"10.1016\/j.mcn.2011.12.001","article-title":"Examination of mesenchymal stem cell-mediated RNAi transfer to Huntington's disease affected neuronal cells for reduction of huntingtin","volume":"49","author":"Olson","year":"2012","journal-title":"Mol. Cell. Neurosci."},{"key":"B37","doi-asserted-by":"publisher","first-page":"163","DOI":"10.1038\/nmeth1104-163","article-title":"Cloning of short hairpin RNAs for gene knockdown in mammalian cells","volume":"1","author":"Paddison","year":"2004","journal-title":"Nat. Methods"},{"key":"B38","doi-asserted-by":"publisher","first-page":"1064","DOI":"10.1038\/nn.3761","article-title":"Transneuronal propagation of mutant huntingtin contributes to non-cell autonomous pathology in neurons","volume":"17","author":"Pecho-Vrieseling","year":"2014","journal-title":"Nat. Neurosci."},{"key":"B39","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1007\/s11920-017-0759-9","article-title":"Juvenile Huntington's disease: diagnostic and treatment considerations for the psychiatrist","volume":"19","author":"Quigley","year":"2017","journal-title":"Curr. Psychiatry Rep."},{"key":"B40","doi-asserted-by":"publisher","first-page":"4319","DOI":"10.1172\/JCI83185","article-title":"Targeting CAG repeat RNAs reduces Huntington's disease phenotype independently of huntingtin levels","volume":"126","author":"Ru\u00e9","year":"2016","journal-title":"J. Clin. Invest."},{"key":"B41","doi-asserted-by":"publisher","first-page":"545","DOI":"10.1038\/nmeth.1209","article-title":"Improving the photostability of bright monomeric orange and red fluorescent proteins","volume":"5","author":"Shaner","year":"2008","journal-title":"Nat. Methods"},{"key":"B42","doi-asserted-by":"publisher","first-page":"1555","DOI":"10.1093\/hmg\/ddg169","article-title":"Selective striatal neuronal loss in a YAC128 mouse model of Huntington disease","volume":"12","author":"Slow","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"B43","doi-asserted-by":"publisher","first-page":"208","DOI":"10.1016\/j.mad.2005.09.012","article-title":"Juvenile Huntington's disease: does a dosage-effect pathogenic mechanism differ from the classical adult disease?","volume":"127","author":"Squitieri","year":"2006","journal-title":"Mech. Ageing Dev."},{"key":"B44","doi-asserted-by":"publisher","first-page":"141","DOI":"10.1016\/S0165-0270(98)00126-5","article-title":"A new method for the rapid and long term growth of human neural precursor cells","volume":"85","author":"Svendsen","year":"1998","journal-title":"J. Neurosci. Methods"},{"key":"B45","doi-asserted-by":"publisher","first-page":"1047","DOI":"10.1242\/dmm.019406","article-title":"Huntington disease iPSCs show early molecular changes in intracellular signaling, the expression of oxidative stress proteins and the p53 pathway","volume":"8","author":"Szlachcic","year":"2015","journal-title":"Dis. Model Mech."},{"key":"B46","doi-asserted-by":"publisher","first-page":"637","DOI":"10.1016\/S1474-4422(13)70088-7","article-title":"Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data","volume":"12","author":"Tabrizi","year":"2013","journal-title":"Lancet Neurol."},{"key":"B47","doi-asserted-by":"publisher","first-page":"9424","DOI":"10.1093\/nar\/gku656","article-title":"5' isomiR variation is of functional and evolutionary importance","volume":"42","author":"Tan","year":"2014","journal-title":"Nucleic Acids Res."},{"key":"B48","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes","volume":"72","year":"1993","journal-title":"Cell"},{"key":"B49","doi-asserted-by":"publisher","first-page":"1929","DOI":"10.1093\/hmg\/ddu609","article-title":"A novel manganese-dependent ATM-p53 signaling pathway is selectively impaired in patient-based neuroprogenitor and murine striatal models of Huntington's disease","volume":"24","author":"Tidball","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"B50","doi-asserted-by":"publisher","first-page":"1513","DOI":"10.1016\/j.bbadis.2016.05.015","article-title":"Nuclear speckles are detention centers for transcripts containing expanded cag repeats","volume":"1862","author":"Urbanek","year":"2016","journal-title":"Biochim. Biophys. Acta"},{"key":"B51","doi-asserted-by":"publisher","first-page":"940","DOI":"10.1016\/j.bbagen.2012.03.017","article-title":"Exosomes: current knowledge of their composition, biological functions, and diagnostic and therapeutic potentials","volume":"1820","author":"Vlassov","year":"2012","journal-title":"Biochim. Biophys. Acta"},{"key":"B52","doi-asserted-by":"publisher","first-page":"7248","DOI":"10.1093\/nar\/gkq601","article-title":"Export of microRNAs and microRNA-protective protein by mammalian cells","volume":"38","author":"Wang","year":"2010","journal-title":"Nucleic Acids Res."},{"key":"B53","doi-asserted-by":"publisher","DOI":"10.1007\/s12035-017-0477-7","article-title":"Huntington disease as a neurodevelopmental disorder and early signs of the disease in stem cells","author":"Wiatr","year":"2017","journal-title":"Mol. Neurobiol"},{"key":"B54","doi-asserted-by":"publisher","first-page":"945","DOI":"10.1016\/j.cell.2014.07.017","article-title":"Single-cell phenotyping within transparent intact tissue through whole-body clearing","volume":"158","author":"Yang","year":"2014","journal-title":"Cell"},{"key":"B55","doi-asserted-by":"publisher","first-page":"363","DOI":"10.1038\/nmeth.1323","article-title":"Generation of transgene-free induced pluripotent mouse stem cells by the piggyBac transposon","volume":"6","author":"Yusa","year":"2009","journal-title":"Nat. Methods"},{"key":"B56","doi-asserted-by":"publisher","first-page":"1531","DOI":"10.1073\/pnas.1008322108","article-title":"A hyperactive piggyBac transposase for mammalian applications","volume":"108","author":"Yusa","year":"2011","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"B57","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1038\/ng1095-155","article-title":"Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue","volume":"11","author":"Zeitlin","year":"1995","journal-title":"Nat. Genet."},{"key":"B58","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1016\/j.brainres.2015.09.020","article-title":"iPSC-based drug screening for Huntington's disease","volume":"1638","author":"Zhang","year":"2015","journal-title":"Brain Res."},{"key":"B59","doi-asserted-by":"publisher","first-page":"459","DOI":"10.1007\/s10571-016-0350-7","article-title":"Potential transfer of polyglutamine and CAG-repeat RNA in extracellular vesicles in Huntington's disease: background and evaluation in cell culture","volume":"36","author":"Zhang","year":"2016","journal-title":"Cell. Mol. Neurobiol."}],"updated-by":[{"DOI":"10.3389\/fnmol.2017.00312","type":"corrigendum","label":"Corrigendum","source":"publisher","updated":{"date-parts":[[2017,9,28]],"date-time":"2017-09-28T00:00:00Z","timestamp":1506556800000}}],"container-title":["Frontiers in Molecular Neuroscience"],"original-title":[],"link":[{"URL":"http:\/\/journal.frontiersin.org\/article\/10.3389\/fnmol.2017.00253\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,10,2]],"date-time":"2019-10-02T03:27:18Z","timestamp":1569986838000},"score":1,"resource":{"primary":{"URL":"http:\/\/journal.frontiersin.org\/article\/10.3389\/fnmol.2017.00253\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,8,8]]},"references-count":59,"alternative-id":["10.3389\/fnmol.2017.00253"],"URL":"https:\/\/doi.org\/10.3389\/fnmol.2017.00253","relation":{"corrigendum":[{"id-type":"doi","id":"10.3389\/fnmol.2017.00312","asserted-by":"object"}]},"ISSN":["1662-5099"],"issn-type":[{"value":"1662-5099","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,8,8]]},"article-number":"253"}}