{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T12:03:13Z","timestamp":1790424193342,"version":"4.1.0"},"reference-count":82,"publisher":"Public Library of Science (PLoS)","issue":"10","license":[{"start":{"date-parts":[[2014,10,9]],"date-time":"2014-10-09T00:00:00Z","timestamp":1412812800000},"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.0109527","type":"journal-article","created":{"date-parts":[[2014,10,9]],"date-time":"2014-10-09T18:13:45Z","timestamp":1412878425000},"page":"e109527","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":55,"title":["Anaplerotic Triheptanoin Diet Enhances Mitochondrial Substrate Use to Remodel the Metabolome and Improve Lifespan, Motor Function, and Sociability in MeCP2-Null Mice"],"prefix":"10.1371","volume":"9","author":[{"given":"Min Jung","family":"Park","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susan","family":"Aja","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qun","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alicia L.","family":"Degano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Penati","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Justin","family":"Zhuo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles R.","family":"Roe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriele V.","family":"Ronnett","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2014,10,9]]},"reference":[{"key":"ref1","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 (New York, NY)"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1093\/hmg\/11.2.115","article-title":"Insight into Rett syndrome: MeCP2 levels display tissue- and cell-specific differences and correlate with neuronal maturation","volume":"11","author":"MD Shahbazian","year":"2002","journal-title":"Human molecular genetics"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1016\/S0387-7604(84)80030-3","article-title":"Rett syndrome\u2013clinical studies and pathophysiological consideration","volume":"6","author":"Y Nomura","year":"1984","journal-title":"Brain & development"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"S50","DOI":"10.1016\/S0387-7604(01)00379-5","article-title":"Neurophysiology of Rett syndrome","volume":"23","author":"Y Nomura","year":"2001","journal-title":"Brain & development"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"S94","DOI":"10.1016\/S0387-7604(01)00352-7","article-title":"Pathophysiology of Rett syndrome from the stand point of clinical characteristics","volume":"23","author":"M Segawa","year":"2001","journal-title":"Brain Dev"},{"key":"ref6","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":"Nature genetics"},{"key":"ref7","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":"Nature genetics"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1542\/peds.91.2.445","article-title":"Epidemiology of Rett syndrome: a population-based registry","volume":"91","author":"CA Kozinetz","year":"1993","journal-title":"Pediatrics"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1007\/BF02723021","article-title":"Developmental disorders of activity dependent neuronal plasticity","volume":"68","author":"MV Johnston","year":"2001","journal-title":"Indian J Pediatr"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"S202","DOI":"10.1016\/S0387-7604(01)00350-3","article-title":"Rett syndrome: clinical correlates of the newly discovered gene","volume":"23","author":"AK Percy","year":"2001","journal-title":"Brain Dev"},{"key":"ref11","article-title":"Mitochondrial dysfunction as a central actor in intellectual disability-related diseases: An overview of Down syndrome, autism, Fragile X and Rett syndrome","author":"D Valenti","year":"2014","journal-title":"Neurosci Biobehav Rev"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"359","DOI":"10.1146\/annurev.genet.39.110304.095751","article-title":"A mitochondrial paradigm of metabolic and degenerative diseases, aging, and cancer: a dawn for evolutionary medicine","volume":"39","author":"DC Wallace","year":"2005","journal-title":"Annual Review of Genetics"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"2533","DOI":"10.1242\/jcs.070490","article-title":"New insights into the role of mitochondria in aging: mitochondrial dynamics and more","volume":"123","author":"AY Seo","year":"2010","journal-title":"Journal of cell science"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1177\/088307389100600216","article-title":"Rett syndrome and mitochondrial enzyme deficiencies","volume":"6","author":"SB Coker","year":"1991","journal-title":"Journal of child neurology"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/0387-7604(93)90045-A","article-title":"Mitochondrial dysfunction in Rett syndrome. An ultrastructural and biochemical study","volume":"15","author":"MT Dotti","year":"1993","journal-title":"Brain & development"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1016\/S0387-7604(88)80010-X","article-title":"Abnormal mitochondria in the Rett syndrome","volume":"10","author":"O Eeg-Olofsson","year":"1988","journal-title":"Brain & development"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1002\/ajmg.1320320131","article-title":"Rett syndrome: genetic clues based on mitochondrial changes in muscle","volume":"32","author":"O Eeg-Olofsson","year":"1989","journal-title":"American Journal of Medical Genetics"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1177\/088307389000500311","article-title":"Rett syndrome: a mitochondrial disease?","volume":"5","author":"O Eeg-Olofsson","year":"1990","journal-title":"Journal of child neurology"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"706","DOI":"10.1111\/j.1365-2265.2008.03386.x","article-title":"Long-term plasma levels of leptin and adiponectin in Rett syndrome","volume":"70","author":"P Blardi","year":"2009","journal-title":"Clinical endocrinology"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/j.jpeds.2006.10.061","article-title":"Rett syndrome and plasma leptin levels","volume":"150","author":"P Blardi","year":"2007","journal-title":"The Journal of pediatrics"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1016\/j.bbr.2010.08.011","article-title":"Progressive motor and respiratory metabolism deficits in post-weaning Mecp2-null male mice","volume":"216","author":"M Pratte","year":"2011","journal-title":"Behavioural brain research"},{"key":"ref22","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":"Molecular and cellular biology"},{"key":"ref23","article-title":"Triheptanoin-A medium chain triglyceride with odd chain fatty acids: A new anaplerotic anticonvulsant treatment?","author":"K Borges","year":"2011","journal-title":"Epilepsy research"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1007\/s10545-006-0290-3","article-title":"Anaplerotic diet therapy in inherited metabolic disease: therapeutic potential","volume":"29","author":"CR Roe","year":"2006","journal-title":"Journal of inherited metabolic disease"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1016\/j.ymgme.2007.07.012","article-title":"Choice of oils for essential fat supplements can enhance production of abnormal metabolites in fat oxidation disorders","volume":"92","author":"CR Roe","year":"2007","journal-title":"Molecular genetics and metabolism"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1007\/s10545-006-0320-1","article-title":"Anaplerotic molecules: current and future","volume":"29","author":"H Brunengraber","year":"2006","journal-title":"Journal of inherited metabolic disease"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"30409","DOI":"10.1074\/jbc.R200006200","article-title":"The key role of anaplerosis and cataplerosis for citric acid cycle function","volume":"277","author":"OE Owen","year":"2002","journal-title":"J Biol Chem"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1016\/j.ymgme.2004.09.007","article-title":"Pyruvate carboxylase deficiency: clinical and biochemical response to anaplerotic diet therapy","volume":"84","author":"F Mochel","year":"2005","journal-title":"Mol Genet Metab"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1016\/j.ymgme.2010.06.017","article-title":"Adult Polyglucosan Body Disease (APBD): Anaplerotic diet therapy (Triheptanoin) and demonstration of defective methylation pathways","volume":"101","author":"CR Roe","year":"2010","journal-title":"Mol Genet Metab"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1172\/JCI0215311","article-title":"Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride","volume":"110","author":"CR Roe","year":"2002","journal-title":"J Clin Invest"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1212\/01.wnl.0000318283.42961.e9","article-title":"Carnitine palmitoyltransferase II deficiency: successful anaplerotic diet therapy","volume":"71","author":"CR Roe","year":"2008","journal-title":"Neurology"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"947","DOI":"10.1016\/j.neuron.2008.07.030","article-title":"Deletion of Mecp2 in Sim1-expressing neurons reveals a critical role for MeCP2 in feeding behavior, aggression, and the response to stress","volume":"59","author":"SL Fyffe","year":"2008","journal-title":"Neuron"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"2626","DOI":"10.1093\/hmg\/ddt111","article-title":"Insulinotropic treatments exacerbate metabolic syndrome in mice lacking MeCP2 function","volume":"22","author":"MR Pitcher","year":"2013","journal-title":"Hum Mol Genet"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"1323","DOI":"10.1172\/JCI115997","article-title":"Increased rate of gluconeogenesis in type II diabetes mellitus. A 13C nuclear magnetic resonance study","volume":"90","author":"I Magnusson","year":"1992","journal-title":"J Clin Invest"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1177\/38.2.1688895","article-title":"Immunohistochemical localization of phosphoenolpyruvate carboxykinase in adult and developing mouse tissues","volume":"38","author":"DB Zimmer","year":"1990","journal-title":"J Histochem Cytochem"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"2389","DOI":"10.1001\/jama.2010.1706","article-title":"Mitochondrial dysfunction in autism","volume":"304","author":"C Giulivi","year":"2010","journal-title":"JAMA: the journal of the American Medical Association"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1016\/j.nbd.2012.06.007","article-title":"Oxidative burden and mitochondrial dysfunction in a mouse model of Rett syndrome","volume":"48","author":"E Grosser","year":"2012","journal-title":"Neurobiol Dis"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1017\/S0012162205000332","article-title":"Mitochondrial dysfunction in autism spectrum disorders: a population-based study","volume":"47","author":"G Oliveira","year":"2005","journal-title":"Dev Med Child Neurol"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1016\/0887-8994(89)90027-1","article-title":"Mitochondrial alterations in Rett syndrome","volume":"5","author":"A Ruch","year":"1989","journal-title":"Pediatric neurology"},{"key":"ref40","first-page":"S21","article-title":"Motor symptoms of the Rett syndrome: abnormal muscle tone, posture, locomotion and stereotyped movement","volume":"14","author":"Y Nomura","year":"1992","journal-title":"Brain & development"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"E724","DOI":"10.1152\/ajpendo.00364.2005","article-title":"Relationship between insulin sensitivity and in vivo mitochondrial function in skeletal muscle","volume":"291","author":"B Sirikul","year":"2006","journal-title":"American journal of physiologyEndocrinology and metabolism"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"E860","DOI":"10.1152\/ajpendo.00366.2005","article-title":"Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats","volume":"291","author":"RP Kinman","year":"2006","journal-title":"Am J Physiol Endocrinol Metab"},{"key":"ref43","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1016\/j.mam.2004.06.002","article-title":"Carnitine acyltransferases and their influence on CoA pools in health and disease","volume":"25","author":"RR Ramsay","year":"2004","journal-title":"Mol Aspects Med"},{"key":"ref44","doi-asserted-by":"crossref","first-page":"S4","DOI":"10.1016\/S0272-6386(03)00112-4","article-title":"The role of carnitine in normal and altered fatty acid metabolism","volume":"41","author":"C Hoppel","year":"2003","journal-title":"Am J Kidney Dis"},{"key":"ref45","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1002\/dmr.5610050307","article-title":"Ketone body metabolism: a physiological and clinical overview","volume":"5","author":"R Nosadini","year":"1989","journal-title":"Diabetes Metab Rev"},{"key":"ref46","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/0005-2728(72)90092-8","article-title":"Ketogenesis in isolated rat liver mitochondria. I. Relationships with the citric acid cycle and with the mitochondrial energy state","author":"M Lopes-Cardozo","year":"1972","journal-title":"Biochim Biophys Acta"},{"key":"ref47","article-title":"A suppressor screen in Mecp2 mutant mice implicates cholesterol metabolism in Rett syndrome","author":"CM Buchovecky","year":"2013","journal-title":"Nat Genet"},{"key":"ref48","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1242\/dmm.011007","article-title":"Preclinical research in Rett syndrome: setting the foundation for translational success","volume":"5","author":"DM Katz","year":"2012","journal-title":"Dis Model Mech"},{"key":"ref49","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1093\/hmg\/dds406","article-title":"Female Mecp2(+\/-) mice display robust behavioral deficits on two different genetic backgrounds providing a framework for pre-clinical studies","volume":"22","author":"RC Samaco","year":"2013","journal-title":"Hum Mol Genet"},{"key":"ref50","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1002\/ajmg.10005","article-title":"Preserved speech variants of the Rett syndrome: molecular and clinical analysis","volume":"104","author":"M Zappella","year":"2001","journal-title":"Am J Med Genet"},{"key":"ref51","doi-asserted-by":"crossref","first-page":"E362","DOI":"10.1152\/ajpendo.00384.2009","article-title":"Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats. II. Effects on lipolysis, glucose production, and liver acyl-CoA profile","volume":"298","author":"L Gu","year":"2010","journal-title":"Am J Physiol Endocrinol Metab"},{"key":"ref52","doi-asserted-by":"crossref","first-page":"27799","DOI":"10.1074\/jbc.M109.048744","article-title":"Interrelations between C4 ketogenesis, C5 ketogenesis, and anaplerosis in the perfused rat liver","volume":"284","author":"S Deng","year":"2009","journal-title":"J Biol Chem"},{"key":"ref53","doi-asserted-by":"crossref","first-page":"9489","DOI":"10.1073\/pnas.85.24.9489","article-title":"ATP sensitizes the insulin receptor to insulin","volume":"85","author":"KD Ridge","year":"1988","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref54","doi-asserted-by":"crossref","first-page":"327","DOI":"10.2337\/diacare.13.3.327","article-title":"PEPCK gene as model of inhibitory effects of insulin on gene transcription","volume":"13","author":"RM O'Brien","year":"1990","journal-title":"Diabetes Care"},{"key":"ref55","doi-asserted-by":"crossref","first-page":"27025","DOI":"10.1074\/jbc.R109.040543","article-title":"What is the metabolic role of phosphoenolpyruvate carboxykinase?","volume":"284","author":"J Yang","year":"2009","journal-title":"J Biol Chem"},{"key":"ref56","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1016\/j.cmet.2007.03.004","article-title":"Cytosolic phosphoenolpyruvate carboxykinase does not solely control the rate of hepatic gluconeogenesis in the intact mouse liver","volume":"5","author":"SC Burgess","year":"2007","journal-title":"Cell Metab"},{"key":"ref57","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1023\/A:1006827601337","article-title":"The mitochondrial permeability transition in toxic, hypoxic and reperfusion injury","volume":"174","author":"JJ Lemasters","year":"1997","journal-title":"Mol Cell Biochem"},{"key":"ref58","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1111\/j.1749-6632.2003.tb07469.x","article-title":"Mitochondrial mechanisms of neural cell death and neuroprotective interventions in Parkinson&apos;s disease","volume":"991","author":"G Fiskum","year":"2003","journal-title":"Ann N Y Acad Sci"},{"key":"ref59","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1161\/01.RES.0000087542.26971.D4","article-title":"Role of the mitochondrial permeability transition in myocardial disease","volume":"93","author":"JN Weiss","year":"2003","journal-title":"Circ Res"},{"key":"ref60","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1042\/BST0340232","article-title":"Calcium, mitochondria and reperfusion injury: a pore way to die","volume":"34","author":"AP Halestrap","year":"2006","journal-title":"Biochem Soc Trans"},{"key":"ref61","doi-asserted-by":"crossref","first-page":"E364","DOI":"10.1152\/ajpendo.00142.2010","article-title":"{beta}-Hydroxybutyrate inhibits insulin-mediated glucose transport in mouse oxidative muscle","volume":"299","author":"T Yamada","year":"2010","journal-title":"Am J Physiol Endocrinol Metab"},{"key":"ref62","doi-asserted-by":"crossref","unstructured":"Haas RH, Rice MA, Trauner DA, Merritt TA (1986) Therapeutic effects of a ketogenic diet in Rett syndrome. Am J Med Genet Suppl 1: 225\u2013246.","DOI":"10.1002\/ajmg.1320250525"},{"key":"ref63","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1177\/08830738030180011801","article-title":"Ketogenic diet in Rett syndrome","volume":"18","author":"GM Liebhaber","year":"2003","journal-title":"J Child Neurol"},{"key":"ref64","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1016\/j.nbd.2010.07.017","article-title":"Anticonvulsant effects of a triheptanoin diet in two mouse chronic seizure models","volume":"40","author":"S Willis","year":"2010","journal-title":"Neurobiol Dis"},{"key":"ref65","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/j.eplepsyres.2012.09.016","article-title":"Triheptanoin reduces seizure susceptibility in a syndrome-specific mouse model of generalized epilepsy","volume":"103","author":"TH Kim","year":"2013","journal-title":"Epilepsy Res"},{"key":"ref66","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1523\/JNEUROSCI.2623-05.2006","article-title":"Learning and memory and synaptic plasticity are impaired in a mouse model of Rett syndrome","volume":"26","author":"P Moretti","year":"2006","journal-title":"J Neurosci"},{"key":"ref67","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1002\/cne.22009","article-title":"Widespread changes in dendritic and axonal morphology in Mecp2-mutant mouse models of Rett syndrome: evidence for disruption of neuronal networks","volume":"514","author":"PV Belichenko","year":"2009","journal-title":"J Comp Neurol"},{"key":"ref68","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1038\/srep00045","article-title":"The short-time structural plasticity of dendritic spines is altered in a model of Rett syndrome","volume":"1","author":"S Landi","year":"2011","journal-title":"Sci Rep"},{"key":"ref69","doi-asserted-by":"crossref","first-page":"468","DOI":"10.1016\/j.biopsych.2005.07.025","article-title":"Postnatal loss of methyl-CpG binding protein 2 in the forebrain is sufficient to mediate behavioral aspects of Rett syndrome in mice","volume":"59","author":"T Gemelli","year":"2006","journal-title":"Biol Psychiatry"},{"key":"ref70","doi-asserted-by":"crossref","first-page":"1604","DOI":"10.1016\/j.bbamcr.2009.07.001","article-title":"Transcriptional coupling of synaptic transmission and energy metabolism: role of nuclear respiratory factor 1 in co-regulating neuronal nitric oxide synthase and cytochrome c oxidase genes in neurons","volume":"1793","author":"SS Dhar","year":"2009","journal-title":"Biochim Biophys Acta"},{"key":"ref71","doi-asserted-by":"crossref","first-page":"S77","DOI":"10.1016\/j.braindev.2004.10.008","article-title":"MeCP2 expression and function during brain development: implications for Rett syndrome&apos;s pathogenesis and clinical evolution","volume":"27","author":"WE Kaufmann","year":"2005","journal-title":"Brain Dev"},{"key":"ref72","doi-asserted-by":"crossref","first-page":"424","DOI":"10.1177\/088307389400900419","article-title":"Neuropathology of Rett syndrome: case report with neuronal and mitochondrial abnormalities in the brain","volume":"9","author":"ME Cornford","year":"1994","journal-title":"J Child Neurol"},{"key":"ref73","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1038\/nature10214","article-title":"A role for glia in the progression of Rett&apos;s syndrome","volume":"475","author":"DT Lioy","year":"2011","journal-title":"Nature"},{"key":"ref74","doi-asserted-by":"crossref","first-page":"1152","DOI":"10.1038\/npp.2011.301","article-title":"Modulation of RhoGTPases improves the behavioral phenotype and reverses astrocytic deficits in a mouse model of Rett syndrome","volume":"37","author":"B De Filippis","year":"2012","journal-title":"Neuropsychopharmacology"},{"key":"ref75","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1002\/ajmg.10633","article-title":"Infantile hypotonia as a presentation of Rett syndrome","volume":"111","author":"HA Heilstedt","year":"2002","journal-title":"American Journal of Medical Genetics"},{"key":"ref76","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1093\/ejechocard\/jer256","article-title":"Subclinical myocardial dysfunction in Rett syndrome","volume":"13","author":"C De Felice","year":"2012","journal-title":"Eur Heart J Cardiovasc Imaging"},{"key":"ref77","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/j.mcn.2014.01.005","article-title":"MeCP2 is required for activity-dependent refinement of olfactory circuits","volume":"59","author":"AL Degano","year":"2014","journal-title":"Mol Cell Neurosci"},{"key":"ref78","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1016\/j.mcn.2009.07.009","article-title":"MeCP2 deficiency disrupts axonal guidance, fasciculation, and targeting by altering Semaphorin 3F function","volume":"42","author":"AL Degano","year":"2009","journal-title":"Molecular and cellular neurosciences"},{"key":"ref79","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":"ref80","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1093\/hmg\/ddi016","article-title":"Abnormalities of social interactions and home-cage behavior in a mouse model of Rett syndrome","volume":"14","author":"P Moretti","year":"2005","journal-title":"Human molecular genetics"},{"key":"ref81","doi-asserted-by":"crossref","first-page":"3270","DOI":"10.1073\/pnas.1019393108","article-title":"Profiling the effects of isocitrate dehydrogenase 1 and 2 mutations on the cellular metabolome","volume":"108","author":"ZJ Reitman","year":"2011","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref82","doi-asserted-by":"crossref","first-page":"6656","DOI":"10.1021\/ac901536h","article-title":"Integrated, nontargeted ultrahigh performance liquid chromatography\/electrospray ionization tandem mass spectrometry platform for the identification and relative quantification of the small-molecule complement of biological systems","volume":"81","author":"AM Evans","year":"2009","journal-title":"Anal Chem"}],"container-title":["PLoS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0109527","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,19]],"date-time":"2022-04-19T18:23:29Z","timestamp":1650392609000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0109527"}},"subtitle":[],"editor":[{"given":"Nicoletta","family":"Landsberger","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2014,10,9]]},"references-count":82,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2014,10,9]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0109527","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2014,10,9]]}}}