scholarly article | Q13442814 |
P819 | ADS bibcode | 2014PLoSO...9j9527P |
P356 | DOI | 10.1371/JOURNAL.PONE.0109527 |
P8608 | Fatcat ID | release_7qivzuh37fagxeb63pub4yxydi |
P3181 | OpenCitations bibliographic resource ID | 2200986 |
P932 | PMC publication ID | 4192301 |
P698 | PubMed publication ID | 25299635 |
P5875 | ResearchGate publication ID | 266682482 |
P50 | author | Min J Park | Q81349913 |
P2093 | author name string | Gabriele V Ronnett | |
Susan Aja | |||
Qun Li | |||
Charles R Roe | |||
Alicia L Degano | |||
Judith Penati | |||
Justin Zhuo | |||
P2860 | cites work | Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2 | Q22337290 |
New insights into the role of mitochondria in aging: mitochondrial dynamics and more | Q24600600 | ||
Cytosolic phosphoenolpyruvate carboxykinase does not solely control the rate of hepatic gluconeogenesis in the intact mouse liver | Q24647278 | ||
MeCP2, a key contributor to neurological disease, activates and represses transcription | Q24647533 | ||
MeCP2 expression and function during brain development: implications for Rett syndrome's pathogenesis and clinical evolution | Q28273844 | ||
A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndrome | Q28504458 | ||
Learning and memory and synaptic plasticity are impaired in a mouse model of Rett syndrome | Q28505346 | ||
Insight into Rett syndrome: MeCP2 levels display tissue- and cell-specific differences and correlate with neuronal maturation | Q28509347 | ||
Postnatal loss of methyl-CpG binding protein 2 in the forebrain is sufficient to mediate behavioral aspects of Rett syndrome in mice | Q28513605 | ||
Gene expression analysis exposes mitochondrial abnormalities in a mouse model of Rett syndrome | Q28592510 | ||
A mitochondrial paradigm of metabolic and degenerative diseases, aging, and cancer: a dawn for evolutionary medicine | Q29547303 | ||
Deficiency of methyl-CpG binding protein-2 in CNS neurons results in a Rett-like phenotype in mice | Q29616328 | ||
Insulinotropic treatments exacerbate metabolic syndrome in mice lacking MeCP2 function | Q30435678 | ||
A suppressor screen in Mecp2 mutant mice implicates cholesterol metabolism in Rett syndrome | Q30442429 | ||
Female Mecp2(+/-) mice display robust behavioral deficits on two different genetic backgrounds providing a framework for pre-clinical studies | Q30445073 | ||
Subclinical myocardial dysfunction in Rett syndrome. | Q51384603 | ||
Rett syndrome and plasma leptin levels. | Q51772628 | ||
Ketogenesis in isolated rat liver mitochondria. I. Relationships with the citric acid cycle and with the mitochondrial energy state. | Q52479465 | ||
Immunohistochemical localization of phosphoenolpyruvate carboxykinase in adult and developing mouse tissues | Q67653522 | ||
Rett syndrome and mitochondrial enzyme deficiencies | Q68239232 | ||
Mitochondrial alterations in Rett syndrome | Q69772944 | ||
Ketogenic diet in Rett syndrome | Q73184781 | ||
Carnitine palmitoyltransferase II deficiency: successful anaplerotic diet therapy | Q30487371 | ||
Preclinical research in Rett syndrome: setting the foundation for translational success. | Q30527828 | ||
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 | Q33485414 | ||
MeCP2 is required for activity-dependent refinement of olfactory circuits | Q33560766 | ||
Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats. II. Effects on lipolysis, glucose production, and liver acyl-CoA profile | Q33655478 | ||
ATP sensitizes the insulin receptor to insulin | Q33684916 | ||
Pyruvate carboxylase deficiency: clinical and biochemical response to anaplerotic diet therapy | Q33985896 | ||
Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats | Q33995907 | ||
The key role of anaplerosis and cataplerosis for citric acid cycle function. | Q34135762 | ||
Neurophysiology of Rett syndrome | Q34460541 | ||
Pathophysiology of Rett syndrome from the stand point of clinical characteristics | Q34460563 | ||
Rett syndrome: clinical correlates of the newly discovered gene | Q34460607 | ||
Profiling the effects of isocitrate dehydrogenase 1 and 2 mutations on the cellular metabolome | Q34602582 | ||
Choice of oils for essential fat supplements can enhance production of abnormal metabolites in fat oxidation disorders | Q34682571 | ||
Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride | Q34793960 | ||
The role of carnitine in normal and altered fatty acid metabolism | Q35128994 | ||
Mitochondrial mechanisms of neural cell death and neuroprotective interventions in Parkinson's disease. | Q35171006 | ||
Role of the mitochondrial permeability transition in myocardial disease | Q35204734 | ||
Increased rate of gluconeogenesis in type II diabetes mellitus. A 13C nuclear magnetic resonance study | Q35606593 | ||
A role for glia in the progression of Rett's syndrome | Q35712588 | ||
MeCP2 deficiency disrupts axonal guidance, fasciculation, and targeting by altering Semaphorin 3F function. | Q35790342 | ||
Carnitine acyltransferases and their influence on CoA pools in health and disease | Q35885439 | ||
Motor symptoms of the Rett syndrome: abnormal muscle tone, posture, locomotion and stereotyped movement | Q36304914 | ||
Calcium, mitochondria and reperfusion injury: a pore way to die. | Q36424289 | ||
Anaplerotic molecules: current and future | Q36501322 | ||
Anaplerotic diet therapy in inherited metabolic disease: therapeutic potential | Q36501326 | ||
The mitochondrial permeability transition in toxic, hypoxic and reperfusion injury | Q36884216 | ||
Deletion of Mecp2 in Sim1-expressing neurons reveals a critical role for MeCP2 in feeding behavior, aggression, and the response to stress. | Q37001615 | ||
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 | Q37348439 | ||
What is the metabolic role of phosphoenolpyruvate carboxykinase? | Q37447862 | ||
Interrelations between C4 ketogenesis, C5 ketogenesis, and anaplerosis in the perfused rat liver | Q37459821 | ||
Mitochondrial dysfunction in autism | Q37558748 | ||
Triheptanoin--a medium chain triglyceride with odd chain fatty acids: a new anaplerotic anticonvulsant treatment? | Q37920144 | ||
PEPCK gene as model of inhibitory effects of insulin on gene transcription | Q38145851 | ||
Mitochondrial dysfunction as a central actor in intellectual disability-related diseases: an overview of Down syndrome, autism, Fragile X and Rett syndrome. | Q38189204 | ||
Ketone body metabolism: a physiological and clinical overview | Q38631639 | ||
The short-time structural plasticity of dendritic spines is altered in a model of Rett syndrome | Q39119937 | ||
Epidemiology of Rett syndrome: a population-based registry. | Q40870783 | ||
Long-term plasma levels of leptin and adiponectin in Rett syndrome. | Q41938870 | ||
Modulation of RhoGTPases improves the behavioral phenotype and reverses astrocytic deficits in a mouse model of Rett syndrome. | Q42132669 | ||
Anticonvulsant effects of a triheptanoin diet in two mouse chronic seizure models | Q42409348 | ||
Adult Polyglucosan Body Disease (APBD): Anaplerotic diet therapy (Triheptanoin) and demonstration of defective methylation pathways | Q42959565 | ||
{beta}-Hydroxybutyrate inhibits insulin-mediated glucose transport in mouse oxidative muscle. | Q43048275 | ||
Mitochondrial dysfunction in autism spectrum disorders: a population-based study. | Q45288693 | ||
Rett syndrome: a mitochondrial disease? | Q45758910 | ||
Widespread changes in dendritic and axonal morphology in Mecp2-mutant mouse models of Rett syndrome: evidence for disruption of neuronal networks | Q46083730 | ||
Therapeutic effects of a ketogenic diet in Rett syndrome | Q46156332 | ||
Triheptanoin reduces seizure susceptibility in a syndrome-specific mouse model of generalized epilepsy. | Q46315193 | ||
Relationship between insulin sensitivity and in vivo mitochondrial function in skeletal muscle | Q46616743 | ||
Neuropathology of Rett syndrome: case report with neuronal and mitochondrial abnormalities in the brain | Q46844441 | ||
Progressive motor and respiratory metabolism deficits in post-weaning Mecp2-null male mice. | Q48088521 | ||
Abnormal mitochondria in the Rett syndrome | Q48144925 | ||
Mitochondrial dysfunction in Rett syndrome. An ultrastructural and biochemical study. | Q48327593 | ||
Oxidative burden and mitochondrial dysfunction in a mouse model of Rett syndrome | Q48454183 | ||
Abnormalities of social interactions and home-cage behavior in a mouse model of Rett syndrome | Q48573215 | ||
Developmental disorders of activity dependent neuronal plasticity | Q48848703 | ||
Rett syndrome--clinical studies and pathophysiological consideration | Q49091531 | ||
Rett syndrome: genetic clues based on mitochondrial changes in muscle | Q50307418 | ||
Infantile hypotonia as a presentation of Rett syndrome | Q50309363 | ||
Preserved speech variants of the Rett syndrome: molecular and clinical analysis | Q50310385 | ||
P275 | copyright license | Creative Commons Attribution 4.0 International | Q20007257 |
P6216 | copyright status | copyrighted | Q50423863 |
P433 | issue | 10 | |
P407 | language of work or name | English | Q1860 |
P921 | main subject | lifetime | Q22675021 |
P304 | page(s) | e109527 | |
P577 | publication date | 2014-10-09 | |
P1433 | published in | PLOS One | Q564954 |
P1476 | title | Anaplerotic triheptanoin diet enhances mitochondrial substrate use to remodel the metabolome and improve lifespan, motor function, and sociability in MeCP2-null mice | |
P478 | volume | 9 |
Q89982713 | Determining the bioenergetic capacity for fatty acid oxidation in the mammalian nervous system |
Q60921105 | High-fat diet accelerates extreme obesity with hyperphagia in female heterozygous Mecp2-null mice |
Q49620553 | How Can a Ketogenic Diet Improve Motor Function? |
Q39164229 | Ketogenic diets improve behaviors associated with autism spectrum disorder in a sex-specific manner in the EL mouse |
Q35995005 | MeCP2 Related Studies Benefit from the Use of CD1 as Genetic Background. |
Q37653680 | Metabolic Dysfunction Underlying Autism Spectrum Disorder and Potential Treatment Approaches |
Q39101800 | Metabolic Dysfunctions in Amyotrophic Lateral Sclerosis Pathogenesis and Potential Metabolic Treatments |
Q37697385 | Mitochondrial Dysfunction in the Pathogenesis of Rett Syndrome: Implications for Mitochondria-Targeted Therapies |
Q46242014 | Mitochondrial fatty acid biosynthesis and muscle fibre plasticity in very long-chain acyl-CoA dehydrogenase-deficient mice |
Q64079076 | Neuronal Redox-Imbalance in Rett Syndrome Affects Mitochondria as Well as Cytosol, and Is Accompanied by Intensified Mitochondrial O Consumption and ROS Release |
Q31115088 | Pentadecanoic and Heptadecanoic Acids: Multifaceted Odd-Chain Fatty Acids |
Q43230803 | RNA sequencing and proteomics approaches reveal novel deficits in the cortex of Mecp2-deficient mice, a model for Rett syndrome |
Q90748674 | Rett Syndrome and CDKL5 Deficiency Disorder: From Bench to Clinic |
Q36273975 | Rett Syndrome: A Focus on Gut Microbiota |
Q64039248 | Senescence Phenomena and Metabolic Alteration in Mesenchymal Stromal Cells from a Mouse Model of Rett Syndrome |
Q48453533 | Suppression of brain cholesterol synthesis in male Mecp2-deficient mice is age dependent and not accompanied by a concurrent change in the rate of fatty acid synthesis |
Q64040183 | Treating Rett syndrome: from mouse models to human therapies |
Q39422277 | Triheptanoin for the treatment of brain energy deficit: A 14-year experience |
Q90619122 | Whole brain delivery of an instability-prone Mecp2 transgene improves behavioral and molecular pathological defects in mouse models of Rett syndrome |
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