Triheptanoin for the treatment of brain energy deficit: A 14-year experience

Respiration-Deficient Astrocytes Survive As Glycolytic Cells In Vivo

(2017) Lotti M. Supplie et al.   JOURNAL OF NEUROSCIENCE

Triheptanoin treatment in patients with pediatric cardiomyopathy associated with long chain-fatty acid oxidation disorders

(2016) J. Vockley et al.   MOLECULAR GENETICS AND METABOLISM

Glial Glycolysis Is Essential for Neuronal Survival in Drosophila

(2015) Anne Volkenhoff et al.   Cell Metabolism

Triheptanoin dramatically reduces paroxysmal motor disorder in patients with GLUT1 deficiency

(2015) Fanny Mochel et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

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

(2015) Jerry Vockley et al.   MOLECULAR GENETICS AND METABOLISM

Triheptanoin improves brain energy metabolism in patients with Huntington disease

(2015) I. M. Adanyeguh et al.   NEUROLOGY

An RNA-Sequencing Transcriptome and Splicing Database of Glia, Neurons, and Vascular Cells of the Cerebral Cortex

(2014) Y. Zhang et al.   JOURNAL OF NEUROSCIENCE

Anaplerotic Triheptanoin Diet Enhances Mitochondrial Substrate Use to Remodel the Metabolome and Improve Lifespan, Motor Function, and Sociability in MeCP2-Null Mice

(2014) Min Jung Park et al.   PLoS One

Triheptanoin for Glucose Transporter Type I Deficiency (G1D)

(2014) Juan M. Pascual et al.   JAMA Neurology

Unsuccessful treatment of severe pyruvate carboxylase deficiency with triheptanoin

(2013) C. Breen et al.   EUROPEAN JOURNAL OF PEDIATRICS

Glut1 Deficiency Syndrome and Novel Ketogenic Diets

(2013) Joerg Klepper et al.   JOURNAL OF CHILD NEUROLOGY

Dietary triheptanoin rescues oligodendrocyte loss, dysmyelination and motor function in the nur7 mouse model of Canavan disease

(2013) Jeremy S. Francis et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Triheptanoin partially restores levels of tricarboxylic acid cycle intermediates in the mouse pilocarpine model of epilepsy

(2013) Mussie G. Hadera et al.   JOURNAL OF NEUROCHEMISTRY

Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data

(2013) Sarah J Tabrizi et al.   LANCET NEUROLOGY

GLUT1 deficiency syndrome: An update

(2013) D. Gras et al.   REVUE NEUROLOGIQUE

Cerebrospinal Fluid Analysis in the Workup of GLUT1 Deficiency Syndrome

(2013) Wilhelmina G. Leen et al.   JAMA Neurology

Heptanoate as a Neural Fuel: Energetic and Neurotransmitter Precursors in Normal and Glucose Transporter I-Deficient (G1D) Brain

(2012) Isaac Marin-Valencia et al.   JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM

Abnormal response to cortical activation in early stages of Huntington disease

(2012) Fanny Mochel et al.   MOVEMENT DISORDERS

Glut1 deficiency (G1D): Epilepsy and metabolic dysfunction in a mouse model of the most common human phenotype

(2012) Isaac Marin-Valencia et al.   NEUROBIOLOGY OF DISEASE

Glut1 deficiency syndrome and erythrocyte glucose uptake assay

(2011) Hong Yang et al.   ANNALS OF NEUROLOGY

GLUT1 deficiency syndrome in clinical practice

(2011) Joerg Klepper   EPILEPSY RESEARCH

Glut1 deficiency: When to suspect and how to diagnose?

(2011) A. Verrotti et al.   EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY

Early Alterations of Brain Cellular Energy Homeostasis in Huntington Disease Models

(2011) Fanny Mochel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Glucose transporter-1 deficiency syndrome: the expanding clinical and genetic spectrum of a treatable disorder

(2010) W. G. Leen et al.   BRAIN

Dietary anaplerotic therapy improves peripheral tissue energy metabolism in patients with Huntington's disease

(2010) Fanny Mochel et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Adult Polyglucosan Body Disease (APBD): Anaplerotic diet therapy (Triheptanoin) and demonstration of defective methylation pathways

(2010) Charles R. Roe et al.   MOLECULAR GENETICS AND METABOLISM

The spectrum of movement disorders in Glut-1 deficiency

(2010) Roser Pons et al.   MOVEMENT DISORDERS

Anticonvulsant effects of a triheptanoin diet in two mouse chronic seizure models

(2010) Sarah Willis et al.   NEUROBIOLOGY OF DISEASE

Parenteral and enteral metabolism of anaplerotic triheptanoin in normal rats. II. Effects on lipolysis, glucose production, and liver acyl-CoA profile

(2009) Lei Gu et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

Interrelations between C4Ketogenesis, C5Ketogenesis, and Anaplerosis in the Perfused Rat Liver

(2009) Shuang Deng et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The metabolic profile of early Huntington's disease- a combined human and transgenic mouse study

(2008) Anna O.G. Goodman et al.   EXPERIMENTAL NEUROLOGY

Carnitine palmitoyltransferase II deficiency: Successful anaplerotic diet therapy

(2008) C. R. Roe et al.   NEUROLOGY

Weight loss in Huntington disease increases with higher CAG repeat number

(2008) N. A. Aziz et al.   NEUROLOGY

Detection of Huntington's disease decades before diagnosis: the Predict-HD study

(2007) J S Paulsen et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY