Medium-Chain Fatty Acids, Beta-Hydroxybutyric Acid and Genetic Modulation of the Carnitine Shuttle Are Protective in a Drosophila Model of ALS Based on TDP-43

The ketogenic diet metabolite beta-hydroxybutyrate (β-HB) reduces incidence of seizure-like activity (SLA) in a K atp - and GABA b -dependent manner in a whole-animal Drosophila melanogaster model

(2017) Jinglu Li et al.   EPILEPSY RESEARCH

Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivo

(2017) Hibiki Kawamata et al.   Molecular Neurodegeneration

Enhancing Mitofusin/Marf ameliorates neuromuscular dysfunction in Drosophila models of TDP-43 proteinopathies

(2017) Bilal Khalil et al.   NEUROBIOLOGY OF AGING

Post-transcriptional Inhibition of Hsc70-4/HSPA8 Expression Leads to Synaptic Vesicle Cycling Defects in Multiple Models of ALS

(2017) Alyssa N. Coyne et al.   Cell Reports

Multi-platform mass spectrometry analysis of the CSF and plasma metabolomes of rigorously matched amyotrophic lateral sclerosis, Parkinson's disease and control subjects

(2016) Anna Wuolikainen et al.   Molecular BioSystems

Alternative Fuels in Epilepsy and Amyotrophic Lateral Sclerosis

(2016) Tesfaye W. Tefera et al.   NEUROCHEMICAL RESEARCH

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

(2016) Tesfaye W. Tefera et al.   PLoS One

Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts

(2016) Elisa Onesto et al.   Acta Neuropathologica Communications

PPAR gamma activation is neuroprotective in a Drosophila model of ALS based on TDP-43

(2014) Archi Joardar et al.   HUMAN MOLECULAR GENETICS

Mitochondrial Dysfunction and Decrease in Body Weight of a Transgenic Knock-in Mouse Model for TDP-43

(2014) Carola Stribl et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Futsch/MAP1B mRNA Is a Translational Target of TDP-43 and Is Neuroprotective in a Drosophila Model of Amyotrophic Lateral Sclerosis

(2014) A. N. Coyne et al.   JOURNAL OF NEUROSCIENCE

Hypercaloric enteral nutrition in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled phase 2 trial

(2014) Anne-Marie Wills et al.   LANCET

A plural role for lipids in motor neuron diseases: energy, signaling and structure

(2014) Florent Schmitt et al.   Frontiers in Cellular Neuroscience

Plasma metabolomic biomarker panel to distinguish patients with amyotrophic lateral sclerosis from disease mimics

(2014) Kay A. Lawton et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Motor neurons and glia exhibit specific individualized responses to TDP-43 expression in a Drosophila model of amyotrophic lateral sclerosis

(2013) P. S. Estes et al.   Disease Models & Mechanisms

The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons

(2013) Wenzhang Wang et al.   HUMAN MOLECULAR GENETICS

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

The changing scene of amyotrophic lateral sclerosis

(2013) Wim Robberecht et al.   NATURE REVIEWS NEUROSCIENCE

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

High-caloric food supplements in the treatment of amyotrophic lateral sclerosis: A prospective interventional study

(2013) Johannes Dorst et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

The genetics and neuropathology of amyotrophic lateral sclerosis

(2012) Ammar Al-Chalabi et al.   ACTA NEUROPATHOLOGICA

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

Biochemical alterations associated with ALS

(2011) Kay A. Lawton et al.   Amyotrophic Lateral Sclerosis

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

(2011) Karin Borges et al.   EPILEPSY RESEARCH

Wild-type and A315T mutant TDP-43 exert differential neurotoxicity in a Drosophila model of ALS

(2011) Patricia S. Estes et al.   HUMAN MOLECULAR GENETICS

Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice

(2011) Ya-Fei Xu et al.   Molecular Neurodegeneration

Impaired glucose tolerance in patients with amyotrophic lateral sclerosis

(2010) Pierre-Francois Pradat et al.   Amyotrophic Lateral Sclerosis

Metabolomic analysis of serum by (1) H NMR spectroscopy in amyotrophic lateral sclerosis

(2010) Alok Kumar et al.   CLINICA CHIMICA ACTA

Energy metabolism in amyotrophic lateral sclerosis

(2010) Luc Dupuis et al.   LANCET NEUROLOGY

1H-NMR-Based Metabolomic Profiling of CSF in Early Amyotrophic Lateral Sclerosis

(2010) Hélène Blasco et al.   PLoS One

Hypermetabolism in ALS patients: an early and persistent phenomenon

(2009) C. Bouteloup et al.   JOURNAL OF NEUROLOGY