Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride
about
Anaplerotic triheptanoin diet enhances mitochondrial substrate use to remodel the metabolome and improve lifespan, motor function, and sociability in MeCP2-null miceHeptanoate as a neural fuel: energetic and neurotransmitter precursors in normal and glucose transporter I-deficient (G1D) brainA review of odd-chain fatty acid metabolism and the role of pentadecanoic Acid (c15:0) and heptadecanoic Acid (c17:0) in health and diseaseMitochondrial dysfunction in fatty acid oxidation disorders: insights from human and animal studiesMitochondrial long chain fatty acid beta-oxidation in man and mouseCardiac hypertrophy in mice with long-chain acyl-CoA dehydrogenase or very long-chain acyl-CoA dehydrogenase deficiencyMedium-chain fatty acids as metabolic therapy in cardiac diseaseTriheptanoin improves brain energy metabolism in patients with Huntington diseaseLong-chain fatty acid combustion rate is associated with unique metabolite profiles in skeletal muscle mitochondriaCarnitine palmitoyltransferase II deficiency: successful anaplerotic diet therapy.Pentadecanoic and Heptadecanoic Acids: Multifaceted Odd-Chain Fatty Acids.Optimal dietary therapy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.Effects of higher dietary protein intake on energy balance and metabolic control in children with long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiency.Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats. II. Effects on lipolysis, glucose production, and liver acyl-CoA profileSerum lipidomics meets cardiac magnetic resonance imaging: profiling of subjects at risk of dilated cardiomyopathy.Inborn errors of energy metabolism associated with myopathies.Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats.Therapies in inborn errors of oxidative metabolism.Sports in LCHAD Deficiency: Maximal Incremental and Endurance Exercise Tests in a 13-Year-Old Patient with Long-Chain 3-Hydroxy Acyl-CoA Dehydrogenase Deficiency (LCHADD) and Heptanoate Treatment.Cardiac anaplerosis in health and disease: food for thought.Triheptanoin for glucose transporter type I deficiency (G1D): modulation of human ictogenesis, cerebral metabolic rate, and cognitive indices by a food supplementClinical outcome, biochemical and therapeutic follow-up in 14 Austrian patients with Long-Chain 3-Hydroxy Acyl CoA Dehydrogenase Deficiency (LCHADD).Comparative study of serine-plasmalogens in human retina and optic nerve: identification of atypical species with odd carbon chains.Compartmentation of Metabolism of the C12-, C9-, and C5-n-dicarboxylates in Rat Liver, Investigated by Mass Isotopomer Analysis: ANAPLEROSIS FROM DODECANEDIOATE.Long-term major clinical outcomes in patients with long chain fatty acid oxidation disorders before and after transition to triheptanoin treatment--A retrospective chart review.Anaplerotic treatment of long-chain fat oxidation disorders with triheptanoin: Review of 15 years ExperienceGenetic basis for correction of very-long-chain acyl-coenzyme A dehydrogenase deficiency by bezafibrate in patient fibroblasts: toward a genotype-based therapyKetone body metabolism and cardiovascular diseaseMolecular and cellular pathology of very-long-chain acyl-CoA dehydrogenase deficiencyUnique plasma metabolomic signatures of individuals with inherited disorders of long-chain fatty acid oxidation.PPARs as therapeutic targets for correction of inborn mitochondrial fatty acid oxidation disorders.Therapeutic options in other metabolic myopathiesMetabolic adaptations to interrupted glycosaminoglycan recycling.Interrelations between C4 ketogenesis, C5 ketogenesis, and anaplerosis in the perfused rat liver.Alterations of hippocampal glucose metabolism by even versus uneven medium chain triglycerides.Medium-Chain Acyl-CoA Dehydrogenase Deficiency in an Infant with Dilated Cardiomyopathy.Current issues regarding treatment of mitochondrial fatty acid oxidation disorders.Triheptanoin--a medium chain triglyceride with odd chain fatty acids: a new anaplerotic anticonvulsant treatment?Genetics and metabolic cardiomyopathies.Rethinking cardiac metabolism: metabolic cycles to refuel and rebuild the failing heart.
P2860
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P2860
Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride
description
2002 nî lūn-bûn
@nan
2002 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
2002 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
2002年の論文
@ja
2002年論文
@yue
2002年論文
@zh-hant
2002年論文
@zh-hk
2002年論文
@zh-mo
2002年論文
@zh-tw
2002年论文
@wuu
name
Treatment of cardiomyopathy an ...... lerotic odd-chain triglyceride
@ast
Treatment of cardiomyopathy an ...... lerotic odd-chain triglyceride
@en
Treatment of cardiomyopathy an ...... lerotic odd-chain triglyceride
@nl
type
label
Treatment of cardiomyopathy an ...... lerotic odd-chain triglyceride
@ast
Treatment of cardiomyopathy an ...... lerotic odd-chain triglyceride
@en
Treatment of cardiomyopathy an ...... lerotic odd-chain triglyceride
@nl
prefLabel
Treatment of cardiomyopathy an ...... lerotic odd-chain triglyceride
@ast
Treatment of cardiomyopathy an ...... lerotic odd-chain triglyceride
@en
Treatment of cardiomyopathy an ...... lerotic odd-chain triglyceride
@nl
P2093
P2860
P356
P1476
Treatment of cardiomyopathy an ...... lerotic odd-chain triglyceride
@en
P2093
Charles R Roe
Diane S Roe
France David
Henri Brunengraber
Lawrence Sweetman
P2860
P304
P356
10.1172/JCI0215311
P407
P577
2002-07-01T00:00:00Z