Triheptanoin Protects Motor Neurons and Delays the Onset of Motor Symptoms in a Mouse Model of Amyotrophic Lateral Sclerosis

Mitochondrial Dysfunction is a Converging Point of Multiple Pathological Pathways in Amyotrophic Lateral Sclerosis

(2017) Ping Shi et al.   JOURNAL OF ALZHEIMERS DISEASE

A metabolic switch toward lipid use in glycolytic muscle is an early pathologic event in a mouse model of amyotrophic lateral sclerosis

(2015) L. Palamiuc et al.   EMBO Molecular Medicine

Triheptanoin improves brain energy metabolism in patients with Huntington disease

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

Gene expression signatures in motor neurone disease fibroblasts reveal dysregulation of metabolism, hypoxia-response and RNA processing functions

(2015) Rohini Raman et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Reduced severity of ischemic stroke and improvement of mitochondrial function after dietary treatment with the anaplerotic substance triheptanoin

(2015) T.M. Schwarzkopf et al.   NEUROSCIENCE

Advances in motor neurone disease

(2014) Dirk Bäumer et al.   JOURNAL OF THE ROYAL SOCIETY OF MEDICINE

Functional pattern of brain FDG-PET in amyotrophic lateral sclerosis

(2014) M. Pagani et al.   NEUROLOGY

Metabolic Therapy with Deanna Protocol Supplementation Delays Disease Progression and Extends Survival in Amyotrophic Lateral Sclerosis (ALS) Mouse Model

(2014) Csilla Ari et al.   PLoS One

Triheptanoin for Glucose Transporter Type I Deficiency (G1D)

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

Triheptanoin Supplementation to Ketogenic Diet Curbs Cognitive Impairment in APP/PS1 Mice Used as a Model of Familial Alzheimer’s Disease

(2013) Ester Aso et al.   Current Alzheimer Research

Alterations of Hippocampal Glucose Metabolism by Even versus Uneven Medium Chain Triglycerides

(2013) Tanya S McDonald et al.   JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM

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

Dysregulation of the complement cascade in the hSOD1G93A transgenic mouse model of amyotrophic lateral sclerosis

(2013) John D Lee et al.   Journal of Neuroinflammation

Genetics of amyotrophic lateral sclerosis: an update

(2013) Sheng Chen et al.   Molecular Neurodegeneration

Defective Mitochondrial Dynamics Is an Early Event in Skeletal Muscle of an Amyotrophic Lateral Sclerosis Mouse Model

(2013) Guo Luo et al.   PLoS One

Gene expression profile of SOD1-G93A mouse spinal cord, blood and muscle

(2013) Christiaan G. J. Saris et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

ALS Untangled No. 20: The Deanna Protocol

(2013)   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

The relationship between Bayesian motor unit number estimation and histological measurements of motor neurons in wild-type and SOD1G93A mice

(2012) S.T. Ngo et al.   CLINICAL NEUROPHYSIOLOGY

Impairments to the GH-IGF-I Axis in hSOD1G93AMice Give Insight into Possible Mechanisms of GH Dysregulation in Patients with Amyotrophic Lateral Sclerosis

(2012) F. J. Steyn et al.   ENDOCRINOLOGY

Triheptanoin in acute mouse seizure models

(2012) Nicola K. Thomas et al.   EPILEPSY RESEARCH

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

Disruption of skeletal muscle mitochondrial network genes and miRNAs in amyotrophic lateral sclerosis

(2012) Aaron P. Russell et al.   NEUROBIOLOGY OF DISEASE

Modulation of Astrocytic Mitochondrial Function by Dichloroacetate Improves Survival and Motor Performance in Inherited Amyotrophic Lateral Sclerosis

(2012) Ernesto Miquel et al.   PLoS One

Caprylic Triglyceride as a Novel Therapeutic Approach to Effectively Improve the Performance and Attenuate the Symptoms Due to the Motor Neuron Loss in ALS Disease

(2012) Wei Zhao et al.   PLoS One

Triheptanoin—A medium chain triglyceride with odd chain fatty acids: A new anaplerotic anticonvulsant treatment?

(2011) Karin Borges et al.   EPILEPSY RESEARCH

Brain hypermetabolism in amyotrophic lateral sclerosis: a FDG PET study in ALS of spinal and bulbar onset

(2011) Angelina Cistaro et al.   EUROPEAN JOURNAL OF NUCLEAR MEDICINE AND MOLECULAR IMAGING

Early and Progressive Impairment of Spinal Blood Flow—Glucose Metabolism Coupling in Motor Neuron Degeneration of ALS Model Mice

(2011) Kazunori Miyazaki et al.   JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM

Amyotrophic lateral sclerosis

(2011) Matthew C Kiernan et al.   LANCET

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

(2011) Laura Ferraiuolo et al.   Nature Reviews Neurology

Glutamate and glutathione interplay in a motor neuronal model of amyotrophic lateral sclerosis reveals altered energy metabolism

(2011) Giuseppina D'Alessandro et al.   NEUROBIOLOGY OF DISEASE

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

Mitochondrial dysfunction in ALS

(2011) Mauro Cozzolino et al.   PROGRESS IN NEUROBIOLOGY

Guidelines for preclinical animal research in ALS/MND: A consensus meeting

(2010) Albert C. Ludolph et al.   Amyotrophic Lateral Sclerosis

Mitochondrial Respiratory Chain Dysfunction in Muscle From Patients With Amyotrophic Lateral Sclerosis

(2010) Veronica Crugnola et al.   ARCHIVES OF NEUROLOGY

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

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

Energy metabolism in amyotrophic lateral sclerosis

(2010) Luc Dupuis et al.   LANCET NEUROLOGY

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

Lactate dyscrasia: a novel explanation for amyotrophic lateral sclerosis

(2010) Sivan Vadakkadath Meethal et al.   NEUROBIOLOGY OF AGING

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

Pathophysiology of Neurodegeneration in Familial Amyotrophic Lateral Sclerosis

(2009) Steve Vucic et al.   CURRENT MOLECULAR MEDICINE

Magnetic resonance spectroscopy of regional brain metabolite markers in FALS mice and the effects of dietary creatine supplementation

(2009) Ji-Kyung Choi et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Oxidative stress in ALS: Key role in motor neuron injury and therapeutic target

(2009) Siân C. Barber et al.   FREE RADICAL BIOLOGY AND MEDICINE

No Benefit from Chronic Lithium Dosing in a Sibling-Matched, Gender Balanced, Investigator-Blinded Trial Using a Standard Mouse Model of Familial ALS

(2009) Alan Gill et al.   PLoS One

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

(2008) Edor Kabashi et al.   NATURE GENETICS

Carnitine palmitoyltransferase II deficiency: Successful anaplerotic diet therapy

(2008) C. R. Roe et al.   NEUROLOGY

Transgenics, toxicity and therapeutics in rodent models of mutant SOD1-mediated familial ALS

(2008) B TURNER et al.   PROGRESS IN NEUROBIOLOGY