{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T22:50:33Z","timestamp":1793141433866,"version":"4.5.0"},"reference-count":44,"publisher":"Public Library of Science (PLoS)","issue":"10","license":[{"start":{"date-parts":[[2011,10,12]],"date-time":"2011-10-12T00:00:00Z","timestamp":1318377600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0026203","type":"journal-article","created":{"date-parts":[[2011,10,12]],"date-time":"2011-10-12T20:58:23Z","timestamp":1318453103000},"page":"e26203","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":281,"title":["Epigenetic Characterization of the FMR1 Gene and Aberrant Neurodevelopment in Human Induced Pluripotent Stem Cell Models of Fragile X Syndrome"],"prefix":"10.1371","volume":"6","author":[{"given":"Steven D.","family":"Sheridan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kraig M.","family":"Theriault","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Surya A.","family":"Reis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fen","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jon M.","family":"Madison","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurence","family":"Daheron","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeanne F.","family":"Loring","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen J.","family":"Haggarty","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2011,10,12]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1146\/annurev.med.60.053107.121225","article-title":"Advances in autism.","volume":"60","author":"DH Geschwind","year":"2009","journal-title":"Annu Rev Med"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"549","DOI":"10.1016\/j.cell.2008.02.010","article-title":"Stem cells and drug discovery: the beginning of a new era?","volume":"132","author":"LL Rubin","year":"2008","journal-title":"Cell"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1136\/jnnp.6.3-4.154","article-title":"A Pedigree of Mental Defect Showing Sex-Linkage.","volume":"6","author":"JP Martin","year":"1943","journal-title":"J Neurol Psychiatry"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1097\/00125817-200109000-00006","article-title":"FMR1 and the fragile X syndrome: human genome epidemiology review.","volume":"3","author":"DC Crawford","year":"2001","journal-title":"Genet Med"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1542\/peds.2008-0317","article-title":"Advances in the treatment of fragile X syndrome.","volume":"123","author":"RJ Hagerman","year":"2009","journal-title":"Pediatrics"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1146\/annurev.genom.8.080706.092249","article-title":"The pathophysiology of fragile x syndrome.","volume":"8","author":"O Penagarikano","year":"2007","journal-title":"Annu Rev Genomics Hum Genet"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1126\/science.7692601","article-title":"FMR1 protein: conserved RNP family domains and selective RNA binding.","volume":"262","author":"CT Ashley Jr","year":"1993","journal-title":"Science"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1016\/0092-8674(93)90420-U","article-title":"The protein product of the fragile X gene, FMR1, has characteristics of an RNA-binding protein.","volume":"74","author":"H Siomi","year":"1993","journal-title":"Cell"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"2648","DOI":"10.1523\/JNEUROSCI.0099-04.2004","article-title":"Metabotropic glutamate receptor activation regulates fragile x mental retardation protein and FMR1 mRNA localization differentially in dendrites and at synapses.","volume":"24","author":"LN Antar","year":"2004","journal-title":"J Neurosci"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"1097","DOI":"10.1126\/science.252.5009.1097","article-title":"Instability of a 550-base pair DNA segment and abnormal methylation in fragile X syndrome.","volume":"252","author":"I Oberle","year":"1991","journal-title":"Science"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"4634","DOI":"10.1093\/hmg\/ddq394","article-title":"The distribution of repressive histone modifications on silenced FMR1 alleles provides clues to the mechanism of gene silencing in fragile X syndrome.","volume":"19","author":"D Kumari","year":"2010","journal-title":"Hum Mol Genet"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"7413","DOI":"10.1074\/jbc.R800026200","article-title":"Chromatin remodeling in the noncoding repeat expansion diseases.","volume":"284","author":"D Kumari","year":"2009","journal-title":"J Biol Chem"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"64ra61","DOI":"10.1126\/scitranslmed.3001708","article-title":"Epigenetic modification of the FMR1 gene in fragile X syndrome is associated with differential response to the mGluR5 antagonist AFQ056.","volume":"3","author":"S Jacquemont","year":"2011","journal-title":"Sci Transl Med"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"1423","DOI":"10.1126\/science.271.5254.1423","article-title":"Friedreich&apos;s ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion.","volume":"271","author":"V Campuzano","year":"1996","journal-title":"Science"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1093\/nar\/23.4.628","article-title":"A transient three-plasmid expression system for the production of high titer retroviral vectors.","volume":"23","author":"Y Soneoka","year":"1995","journal-title":"Nucleic Acids Res"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1038\/nature06534","article-title":"Reprogramming of human somatic cells to pluripotency with defined factors.","volume":"451","author":"IH Park","year":"2008","journal-title":"Nature"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/j.bbrc.2010.01.101","article-title":"Propagation of human embryonic and induced pluripotent stem cells in an indirect co-culture system.","volume":"393","author":"S Abraham","year":"2010","journal-title":"Biochem Biophys Res Commun"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/S0091-679X(08)00003-4","article-title":"Microporous membrane growth substrates for embryonic stem cell culture and differentiation.","volume":"86","author":"SD Sheridan","year":"2008","journal-title":"Methods Cell Biol"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"568","DOI":"10.1016\/j.stem.2007.09.001","article-title":"Developmental study of fragile X syndrome using human embryonic stem cells derived from preimplantation genetically diagnosed embryos.","volume":"1","author":"R Eiges","year":"2007","journal-title":"Cell Stem Cell"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/j.ymeth.2008.03.005","article-title":"Protocols for cytogenetic studies of human embryonic stem cells.","volume":"45","author":"LF Meisner","year":"2008","journal-title":"Methods"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1097\/00125817-200105000-00010","article-title":"Technical standards and guidelines for fragile X: the first of a series of disease-specific supplements to the Standards and Guidelines for Clinical Genetics Laboratories of the American College of Medical Genetics. Quality Assurance Subcommittee of the Laboratory Practice Committee.","volume":"3","author":"A Maddalena","year":"2001","journal-title":"Genetics in medicine : official journal of the American College of Medical Genetics"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"152","DOI":"10.2144\/03351md02","article-title":"Analysis and quantification of multiple methylation variable positions in CpG islands by Pyrosequencing.","volume":"35","author":"J Tost","year":"2003","journal-title":"Biotechniques"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1373\/clinchem.2007.072629","article-title":"Quantitative high-resolution CpG island mapping with Pyrosequencing reveals disease-specific methylation patterns of the CDKN2B gene in myelodysplastic syndrome and myeloid leukemia.","volume":"53","author":"K Brakensiek","year":"2007","journal-title":"Clin Chem"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1002\/ajmg.1320510444","article-title":"Mosaicism in fragile X affected males.","volume":"51","author":"SL Nolin","year":"1994","journal-title":"Am J Med Genet"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1093\/hmg\/1.6.397","article-title":"DNA methylation represses FMR-1 transcription in fragile X syndrome.","volume":"1","author":"JS Sutcliffe","year":"1992","journal-title":"Hum Mol Genet"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1086\/302720","article-title":"Elevated levels of FMR1 mRNA in carrier males: a new mechanism of involvement in the fragile-X syndrome.","volume":"66","author":"F Tassone","year":"2000","journal-title":"Am J Hum Genet"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1126\/science.7732383","article-title":"Translational suppression by trinucleotide repeat expansion at FMR1.","volume":"268","author":"Y Feng","year":"1995","journal-title":"Science"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1016\/j.cell.2006.07.024","article-title":"Induction of pluripotent stem cells from mouse embryonic and adult fibroblast cultures by defined factors.","volume":"126","author":"K Takahashi","year":"2006","journal-title":"Cell"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1016\/j.stem.2008.11.008","article-title":"Guidelines and techniques for the generation of induced pluripotent stem cells.","volume":"3","author":"N Maherali","year":"2008","journal-title":"Cell Stem Cell"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1007\/BF03401776","article-title":"Differentiation of human embryonic stem cells into embryoid bodies compromising the three embryonic germ layers.","volume":"6","author":"J Itskovitz-Eldor","year":"2000","journal-title":"Mol Med"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"17834","DOI":"10.1073\/pnas.0508995102","article-title":"Altered differentiation of neural stem cells in fragile X syndrome.","volume":"102","author":"M Castren","year":"2005","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1089\/scd.2007.0073","article-title":"Normal Neurogenesis but Abnormal Gene Expression in Human Fragile X Cortical Progenitor Cells.","volume":"17","author":"A Bhattacharyya","year":"2008","journal-title":"Stem Cells Dev"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1016\/S0140-6736(07)60111-1","article-title":"Huntington&apos;s disease.","volume":"369","author":"FO Walker","year":"2007","journal-title":"Lancet"},{"key":"ref34","first-page":"1061","article-title":"The Friedreich ataxia critical region spans a 150-kb interval on chromosome 9q13.","volume":"57","author":"L Montermini","year":"1995","journal-title":"American journal of human genetics"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"1693","DOI":"10.1093\/hmg\/10.16.1693","article-title":"Instability of a (CGG)98 repeat in the Fmr1 promoter.","volume":"10","author":"CJ Bontekoe","year":"2001","journal-title":"Hum Mol Genet"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1159\/000484780","article-title":"FMR1 premutation allele (CGG)81 is stable in mice.","volume":"5","author":"CJ Bontekoe","year":"1997","journal-title":"Eur J Hum Genet"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"24325","DOI":"10.1074\/jbc.271.40.24325","article-title":"Nucleosome assembly on methylated CGG triplet repeats in the fragile X mental retardation gene 1 promoter.","volume":"271","author":"JS Godde","year":"1996","journal-title":"J Biol Chem"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1007\/s10577-011-9206-7","article-title":"Nucleosomal occupancy and CGG repeat expansion: a comparative analysis of triplet repeat region from mouse and human fragile X mental retardation gene 1.","volume":"19","author":"S Datta","year":"2011","journal-title":"Chromosome Res"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"504","DOI":"10.1086\/322739","article-title":"Demethylation, reactivation, and destabilization of human fragile X full-mutation alleles in mouse embryocarcinoma cells.","volume":"69","author":"D Wohrle","year":"2001","journal-title":"Am J Hum Genet"},{"key":"ref40","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/j.stem.2010.04.005","article-title":"Differential modeling of fragile X syndrome by human embryonic stem cells and induced pluripotent stem cells.","volume":"6","author":"A Urbach","year":"2010","journal-title":"Cell Stem Cell"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"631","DOI":"10.1016\/j.stem.2010.09.014","article-title":"Friedreich&apos;s ataxia induced pluripotent stem cells model intergenerational GAATTC triplet repeat instability.","volume":"7","author":"S Ku","year":"2010","journal-title":"Cell Stem Cell"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"1045","DOI":"10.1093\/cercor\/10.10.1045","article-title":"FMRP involvement in formation of synapses among cultured hippocampal neurons.","volume":"10","author":"K Braun","year":"2000","journal-title":"Cereb Cortex"},{"key":"ref43","doi-asserted-by":"crossref","first-page":"e1000898","DOI":"10.1371\/journal.pgen.1000898","article-title":"Fragile x mental retardation protein regulates proliferation and differentiation of adult neural stem\/progenitor cells.","volume":"6","author":"Y Luo","year":"2010","journal-title":"PLoS Genet"},{"key":"ref44","doi-asserted-by":"crossref","first-page":"1528","DOI":"10.4161\/cc.5.14.3096","article-title":"Differentiation of neuronal cells in fragile X syndrome.","volume":"5","author":"M Castren","year":"2006","journal-title":"Cell Cycle"}],"container-title":["PLoS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0026203","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,6,25]],"date-time":"2020-06-25T22:34:07Z","timestamp":1593124447000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0026203"}},"subtitle":[],"editor":[{"given":"Mark R.","family":"Cookson","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2011,10,12]]},"references-count":44,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2011,10,12]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0026203","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,10,12]]}}}