{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T06:49:40Z","timestamp":1789454980747,"version":"build-2803163510"},"reference-count":58,"publisher":"Public Library of Science (PLoS)","issue":"9","license":[{"start":{"date-parts":[[2008,9,5]],"date-time":"2008-09-05T00:00:00Z","timestamp":1220572800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosgenetics.org"],"crossmark-restriction":false},"short-container-title":["PLoS Genet"],"DOI":"10.1371\/journal.pgen.1000179","type":"journal-article","created":{"date-parts":[[2008,9,4]],"date-time":"2008-09-04T21:58:03Z","timestamp":1220565483000},"page":"e1000179","update-policy":"https:\/\/doi.org\/10.1371\/journal.pgen.corrections_policy","source":"Crossref","is-referenced-by-count":71,"title":["Genetic Modifiers of MeCP2 Function in Drosophila"],"prefix":"10.1371","volume":"4","author":[{"given":"Holly N.","family":"Cukier","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alma M.","family":"Perez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ann L.","family":"Collins","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhaolan","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huda Y.","family":"Zoghbi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan","family":"Botas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2008,9,5]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1016\/j.neuron.2007.10.001","article-title":"The story of Rett syndrome: from clinic to neurobiology.","volume":"56","author":"M Chahrour","year":"2007","journal-title":"Neuron"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1038\/13810","article-title":"Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2.","volume":"23","author":"RE Amir","year":"1999","journal-title":"Nat Genet"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1016\/S0887-8994(02)00624-0","article-title":"Identification of MeCP2 mutations in a series of females with autistic disorder.","volume":"28","author":"RM Carney","year":"2003","journal-title":"Pediatr Neurol"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"e12","DOI":"10.1136\/jmg.2004.023804","article-title":"Submicroscopic duplication in Xq28 causes increased expression of the MECP2 gene in a boy with severe mental retardation and features of Rett syndrome.","volume":"42","author":"M Meins","year":"2005","journal-title":"J Med Genet"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1086\/444549","article-title":"Duplication of the MECP2 region is a frequent cause of severe mental retardation and progressive neurological symptoms in males.","volume":"77","author":"H Van Esch","year":"2005","journal-title":"Am J Hum Genet"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"784","DOI":"10.1097\/01.gim.0000250502.28516.3c","article-title":"Increased MECP2 gene copy number as the result of genomic duplication in neurodevelopmentally delayed males.","volume":"8","author":"D Del Gaudio","year":"2006","journal-title":"Genet Med"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1038\/85906","article-title":"Deficiency of methyl-CpG binding protein-2 in CNS neurons results in a Rett-like phenotype in mice.","volume":"27","author":"RZ Chen","year":"2001","journal-title":"Nat Genet"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1038\/85899","article-title":"A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndrome.","volume":"27","author":"J Guy","year":"2001","journal-title":"Nat Genet"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"2679","DOI":"10.1093\/hmg\/ddh282","article-title":"Mild overexpression of MeCP2 causes a progressive neurological disorder in mice.","volume":"13","author":"AL Collins","year":"2004","journal-title":"Hum Mol Genet"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"6033","DOI":"10.1073\/pnas.0401626101","article-title":"Expression of MeCP2 in postmitotic neurons rescues Rett syndrome in mice.","volume":"101","author":"S Luikenhuis","year":"2004","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"4886","DOI":"10.1093\/nar\/21.21.4886","article-title":"Dissection of the methyl-CpG binding domain from the chromosomal protein MeCP2.","volume":"21","author":"X Nan","year":"1993","journal-title":"Nucleic Acids Res"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"414","DOI":"10.1128\/MCB.16.1.414","article-title":"DNA methylation specifies chromosomal localization of MeCP2.","volume":"16","author":"X Nan","year":"1996","journal-title":"Mol Cell Biol"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1016\/S0168-9525(03)00080-5","article-title":"The methyl-CpG binding domain and the evolving role of DNA methylation in animals.","volume":"19","author":"B Hendrich","year":"2003","journal-title":"Trends Genet"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1038\/30764","article-title":"Transcriptional repression by the methyl-CpG-binding protein MeCP2 involves a histone deacetylase complex.","volume":"393","author":"X Nan","year":"1998","journal-title":"Nature"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"1747","DOI":"10.1126\/science.1076469","article-title":"Corepressor-dependent silencing of chromosomal regions encoding neuronal genes.","volume":"298","author":"VV Lunyak","year":"2002","journal-title":"Science"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"254","DOI":"10.1038\/ng1516","article-title":"Brahma links the SWI\/SNF chromatin-remodeling complex with MeCP2-dependent transcriptional silencing.","volume":"37","author":"KN Harikrishnan","year":"2005","journal-title":"Nat Genet"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1146\/annurev.genet.38.072902.091907","article-title":"Epigenetic regulation of cellular memory by the Polycomb and Trithorax group proteins.","volume":"38","author":"L Ringrose","year":"2004","journal-title":"Annu Rev Genet"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1146\/annurev.biochem.75.103004.142422","article-title":"Chromatin modifications by methylation and ubiquitination: implications in the regulation of gene expression.","volume":"75","author":"A Shilatifard","year":"2006","journal-title":"Annu Rev Biochem"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"14372","DOI":"10.1073\/pnas.96.25.14372","article-title":"Mammalian Trithorax and polycomb-group homologues are antagonistic regulators of homeotic development.","volume":"96","author":"RD Hanson","year":"1999","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1016\/S0092-8674(03)00157-0","article-title":"Polycomb, epigenomes, and control of cell identity.","volume":"112","author":"V Orlando","year":"2003","journal-title":"Cell"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1038\/nrg1981","article-title":"Polycomb silencing mechanisms and the management of genomic programmes.","volume":"8","author":"YB Schwartz","year":"2007","journal-title":"Nat Rev Genet"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"4172","DOI":"10.1093\/nar\/28.21.4172","article-title":"Functional consequences of Rett syndrome mutations on human MeCP2.","volume":"28","author":"TM Yusufzai","year":"2000","journal-title":"Nucleic Acids Res"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1242\/dev.118.2.401","article-title":"Targeted gene expression as a means of altering cell fates and generating dominant phenotypes.","volume":"118","author":"AH Brand","year":"1993","journal-title":"Development"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"855","DOI":"10.1242\/dev.119.3.855","article-title":"Expression of Drosophila glass protein and evidence for negative regulation of its activity in non-neuronal cells by another DNA-binding protein.","volume":"119","author":"MC Ellis","year":"1993","journal-title":"Development"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1016\/j.neuron.2006.09.037","article-title":"Brain-specific phosphorylation of MeCP2 regulates activity-dependent Bdnf transcription, dendritic growth, and spine maturation.","volume":"52","author":"Z Zhou","year":"2006","journal-title":"Neuron"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/S1567-133X(01)00011-4","article-title":"Drosophila cholinergic neurons and processes visualized with Gal4\/UAS-GFP.","volume":"1","author":"PM Salvaterra","year":"2001","journal-title":"Brain Res Gene Expr Patterns"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"e82","DOI":"10.1371\/journal.pgen.0030082","article-title":"Huntingtin interacting proteins are genetic modifiers of neurodegeneration.","volume":"3","author":"LS Kaltenbach","year":"2007","journal-title":"PLoS Genet"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1007\/s004270050212","article-title":"The Drosophila Sin3 gene encodes a widely distributed transcription factor essential for embryonic viability.","volume":"208","author":"G Pennetta","year":"1998","journal-title":"Dev Genes Evol"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/S1097-2765(00)80365-2","article-title":"SMRTER, a Drosophila nuclear receptor coregulator, reveals that EcR-mediated repression is critical for development.","volume":"4","author":"CC Tsai","year":"1999","journal-title":"Mol Cell"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1038\/ng0197-74","article-title":"De novo truncating mutations in E6-AP ubiquitin-protein ligase gene (UBE3A) in Angelman syndrome.","volume":"15","author":"T Matsuura","year":"1997","journal-title":"Nat Genet"},{"key":"ref31","article-title":"Expression of the Rho-GEF Pbl\/ECT2 is regulated by the UBE3A E3 ubiquitin ligase.","author":"LT Reiter","year":"2006","journal-title":"Hum Mol Genet"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1038\/35040584","article-title":"Identification of genes that modify ataxin-1-induced neurodegeneration.","volume":"408","author":"P Fernandez-Funez","year":"2000","journal-title":"Nature"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1093\/hmg\/ddm315","article-title":"Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases.","volume":"17","author":"J Branco","year":"2008","journal-title":"Hum Mol Genet"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"864","DOI":"10.1128\/MCB.01593-06","article-title":"Multiple modes of interaction between the methylated DNA binding protein MeCP2 and chromatin.","volume":"27","author":"T Nikitina","year":"2007","journal-title":"Mol Cell Biol"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1016\/S0959-437X(99)80024-0","article-title":"DNA methylation and chromatin modification.","volume":"9","author":"HH Ng","year":"1999","journal-title":"Curr Opin Genet Dev"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"1765","DOI":"10.1093\/nar\/gkg249","article-title":"The affinity of different MBD proteins for a specific methylated locus depends on their intrinsic binding properties.","volume":"31","author":"MF Fraga","year":"2003","journal-title":"Nucleic Acids Res"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"5656","DOI":"10.1128\/MCB.17.9.5656","article-title":"Chicken MAR-binding protein ARBP is homologous to rat methyl-CpG-binding protein MeCP2.","volume":"17","author":"JM Weitzel","year":"1997","journal-title":"Mol Cell Biol"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1016\/j.molcel.2005.07.021","article-title":"DNA binding selectivity of MeCP2 due to a requirement for A\/T sequences adjacent to methyl-CpG.","volume":"19","author":"RJ Klose","year":"2005","journal-title":"Mol Cell"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"32181","DOI":"10.1074\/jbc.M305308200","article-title":"Chromatin compaction by human MeCP2. Assembly of novel secondary chromatin structures in the absence of DNA methylation.","volume":"278","author":"PT Georgel","year":"2003","journal-title":"J Biol Chem"},{"key":"ref40","doi-asserted-by":"crossref","first-page":"19416","DOI":"10.1073\/pnas.0707442104","article-title":"Integrated epigenomic analyses of neuronal MeCP2 reveal a role for long-range interaction with active genes.","volume":"104","author":"DH Yasui","year":"2007","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"1224","DOI":"10.1126\/science.1153252","article-title":"MeCP2, a key contributor to neurological disease, activates and represses transcription.","volume":"320","author":"M Chahrour","year":"2008","journal-title":"Science"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1038\/35046205","article-title":"DNA methylation in Drosophila melanogaster.","volume":"408","author":"F Lyko","year":"2000","journal-title":"Nature"},{"key":"ref43","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1016\/j.cell.2004.09.036","article-title":"Control of dendritic branching and tiling by the Tricornered-kinase\/Furry signaling pathway in Drosophila sensory neurons.","volume":"119","author":"K Emoto","year":"2004","journal-title":"Cell"},{"key":"ref44","doi-asserted-by":"crossref","first-page":"45773","DOI":"10.1074\/jbc.M403552200","article-title":"Neuronal functions of the novel serine\/threonine kinase Ndr2.","volume":"279","author":"O Stork","year":"2004","journal-title":"J Biol Chem"},{"key":"ref45","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1136\/jmg.38.4.224","article-title":"Angelman syndrome phenotype associated with mutations in MECP2, a gene encoding a methyl CpG binding protein.","volume":"38","author":"P Watson","year":"2001","journal-title":"J Med Genet"},{"key":"ref46","doi-asserted-by":"crossref","first-page":"355","DOI":"10.1016\/j.pediatrneurol.2004.12.012","article-title":"Another patient with MECP2 mutation without classic Rett syndrome phenotype.","volume":"32","author":"D Milani","year":"2005","journal-title":"Pediatr Neurol"},{"key":"ref47","doi-asserted-by":"crossref","first-page":"1049","DOI":"10.1093\/hmg\/ddi097","article-title":"MeCP2 deficiency in Rett syndrome causes epigenetic aberrations at the PWS\/AS imprinting center that affects UBE3A expression.","volume":"14","author":"K Makedonski","year":"2005","journal-title":"Hum Mol Genet"},{"key":"ref48","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1093\/hmg\/ddi045","article-title":"Epigenetic overlap in autism-spectrum neurodevelopmental disorders: MECP2 deficiency causes reduced expression of UBE3A and GABRB3.","volume":"14","author":"RC Samaco","year":"2005","journal-title":"Hum Mol Genet"},{"key":"ref49","doi-asserted-by":"crossref","first-page":"2210","DOI":"10.1093\/hmg\/ddl146","article-title":"Ube3a expression is not altered in Mecp2 mutant mice.","volume":"15","author":"C Jordan","year":"2006","journal-title":"Hum Mol Genet"},{"key":"ref50","doi-asserted-by":"crossref","first-page":"R221","DOI":"10.1093\/hmg\/ddg286","article-title":"DNA methylation and Rett syndrome.","volume":"12 Spec No 2","author":"S Kriaucionis","year":"2003","journal-title":"Hum Mol Genet"},{"key":"ref51","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1177\/1073858403260995","article-title":"Rett syndrome: a prototypical neurodevelopmental disorder.","volume":"10","author":"JL Neul","year":"2004","journal-title":"Neuroscientist"},{"key":"ref52","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1006\/nbdi.2001.0428","article-title":"Gene expression profiling in postmortem Rett Syndrome brain: differential gene expression and patient classification.","volume":"8","author":"C Colantuoni","year":"2001","journal-title":"Neurobiol Dis"},{"key":"ref53","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1186\/1471-2350-3-12","article-title":"Gene expression patterns vary in clonal cell cultures from Rett syndrome females with eight different MECP2 mutations.","volume":"3","author":"J Traynor","year":"2002","journal-title":"BMC Med Genet"},{"key":"ref54","doi-asserted-by":"crossref","first-page":"2247","DOI":"10.1093\/hmg\/ddi229","article-title":"Up-regulation of glucocorticoid-regulated genes in a mouse model of Rett syndrome.","volume":"14","author":"UA Nuber","year":"2005","journal-title":"Hum Mol Genet"},{"key":"ref55","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1186\/1471-2350-7-61","article-title":"Expression profiling of clonal lymphocyte cell cultures from Rett syndrome patients.","volume":"7","author":"IJ Delgado","year":"2006","journal-title":"BMC Med Genet"},{"key":"ref56","doi-asserted-by":"crossref","first-page":"5033","DOI":"10.1128\/MCB.01665-05","article-title":"Gene expression analysis exposes mitochondrial abnormalities in a mouse model of Rett syndrome.","volume":"26","author":"S Kriaucionis","year":"2006","journal-title":"Mol Cell Biol"},{"key":"ref57","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1186\/1471-2350-8-36","article-title":"Cerebellar gene expression profiles of mouse models for Rett syndrome reveal novel MeCP2 targets.","volume":"8","author":"C Jordan","year":"2007","journal-title":"BMC Med Genet"},{"key":"ref58","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1002\/ajmg.10557","article-title":"Balanced X chromosome inactivation patterns in the Rett syndrome brain.","volume":"111","author":"MD Shahbazian","year":"2002","journal-title":"Am J Med Genet"}],"container-title":["PLoS Genetics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pgen.1000179","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,9,16]],"date-time":"2021-09-16T01:19:46Z","timestamp":1631755186000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pgen.1000179"}},"subtitle":[],"editor":[{"given":"Harry","family":"Orr","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2008,9,5]]},"references-count":58,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2008,9,5]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pgen.1000179","relation":{},"ISSN":["1553-7404"],"issn-type":[{"value":"1553-7404","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,9,5]]}}}