Anaplerotic triheptanoin diet enhances mitochondrial substrate use to remodel the metabolome and improve lifespan, motor function, and sociability in MeCP2-null mice

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Anaplerotic triheptanoin diet enhances mitochondrial substrate use to remodel the metabolome and improve lifespan, motor function, and sociability in MeCP2-null mice is …
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scholarly articleQ13442814

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P819ADS bibcode2014PLoSO...9j9527P
P356DOI10.1371/JOURNAL.PONE.0109527
P8608Fatcat IDrelease_7qivzuh37fagxeb63pub4yxydi
P3181OpenCitations bibliographic resource ID2200986
P932PMC publication ID4192301
P698PubMed publication ID25299635
P5875ResearchGate publication ID266682482

P50authorMin J ParkQ81349913
P2093author name stringGabriele V Ronnett
Susan Aja
Qun Li
Charles R Roe
Alicia L Degano
Judith Penati
Justin Zhuo
P2860cites workRett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2Q22337290
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A mouse Mecp2-null mutation causes neurological symptoms that mimic Rett syndromeQ28504458
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Gene expression analysis exposes mitochondrial abnormalities in a mouse model of Rett syndromeQ28592510
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Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats. II. Effects on lipolysis, glucose production, and liver acyl-CoA profileQ33655478
ATP sensitizes the insulin receptor to insulinQ33684916
Pyruvate carboxylase deficiency: clinical and biochemical response to anaplerotic diet therapyQ33985896
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Choice of oils for essential fat supplements can enhance production of abnormal metabolites in fat oxidation disordersQ34682571
Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglycerideQ34793960
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Mitochondrial mechanisms of neural cell death and neuroprotective interventions in Parkinson's disease.Q35171006
Role of the mitochondrial permeability transition in myocardial diseaseQ35204734
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A role for glia in the progression of Rett's syndromeQ35712588
MeCP2 deficiency disrupts axonal guidance, fasciculation, and targeting by altering Semaphorin 3F function.Q35790342
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Motor symptoms of the Rett syndrome: abnormal muscle tone, posture, locomotion and stereotyped movementQ36304914
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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 neuronsQ37348439
What is the metabolic role of phosphoenolpyruvate carboxykinase?Q37447862
Interrelations between C4 ketogenesis, C5 ketogenesis, and anaplerosis in the perfused rat liverQ37459821
Mitochondrial dysfunction in autismQ37558748
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 transcriptionQ38145851
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 overviewQ38631639
The short-time structural plasticity of dendritic spines is altered in a model of Rett syndromeQ39119937
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 modelsQ42409348
Adult Polyglucosan Body Disease (APBD): Anaplerotic diet therapy (Triheptanoin) and demonstration of defective methylation pathwaysQ42959565
{beta}-Hydroxybutyrate inhibits insulin-mediated glucose transport in mouse oxidative muscle.Q43048275
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Widespread changes in dendritic and axonal morphology in Mecp2-mutant mouse models of Rett syndrome: evidence for disruption of neuronal networksQ46083730
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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 muscleQ46616743
Neuropathology of Rett syndrome: case report with neuronal and mitochondrial abnormalities in the brainQ46844441
Progressive motor and respiratory metabolism deficits in post-weaning Mecp2-null male mice.Q48088521
Abnormal mitochondria in the Rett syndromeQ48144925
Mitochondrial dysfunction in Rett syndrome. An ultrastructural and biochemical study.Q48327593
Oxidative burden and mitochondrial dysfunction in a mouse model of Rett syndromeQ48454183
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Rett syndrome--clinical studies and pathophysiological considerationQ49091531
Rett syndrome: genetic clues based on mitochondrial changes in muscleQ50307418
Infantile hypotonia as a presentation of Rett syndromeQ50309363
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P275copyright licenseCreative Commons Attribution 4.0 InternationalQ20007257
P6216copyright statuscopyrightedQ50423863
P433issue10
P407language of work or nameEnglishQ1860
P921main subjectlifetimeQ22675021
P304page(s)e109527
P577publication date2014-10-09
P1433published inPLOS OneQ564954
P1476titleAnaplerotic triheptanoin diet enhances mitochondrial substrate use to remodel the metabolome and improve lifespan, motor function, and sociability in MeCP2-null mice
P478volume9

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cites work (P2860)
Q89982713Determining the bioenergetic capacity for fatty acid oxidation in the mammalian nervous system
Q60921105High-fat diet accelerates extreme obesity with hyperphagia in female heterozygous Mecp2-null mice
Q49620553How Can a Ketogenic Diet Improve Motor Function?
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Q35995005MeCP2 Related Studies Benefit from the Use of CD1 as Genetic Background.
Q37653680Metabolic Dysfunction Underlying Autism Spectrum Disorder and Potential Treatment Approaches
Q39101800Metabolic Dysfunctions in Amyotrophic Lateral Sclerosis Pathogenesis and Potential Metabolic Treatments
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Q31115088Pentadecanoic and Heptadecanoic Acids: Multifaceted Odd-Chain Fatty Acids
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Q39422277Triheptanoin for the treatment of brain energy deficit: A 14-year experience
Q90619122Whole brain delivery of an instability-prone Mecp2 transgene improves behavioral and molecular pathological defects in mouse models of Rett syndrome

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