Genetically altering organismal metabolism by leptin-deficiency benefits a mouse model of amyotrophic lateral sclerosis

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

Diet-Induced Developmental Acceleration Independent of TOR and Insulin in C. elegans

(2013) Lesley T. MacNeil et al.   CELL

High-fat and high-sucrose (western) diet induces steatohepatitis that is dependent on fructokinase

(2013) Takuji Ishimoto et al.   HEPATOLOGY

PGC-1  is a male-specific disease modifier of human and experimental amyotrophic lateral sclerosis

(2013) J. Eschbach et al.   HUMAN MOLECULAR GENETICS

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

(2013) Shuo-Chien Ling et al.   NEURON

Effect of genetic background on onset and disease progression in the SOD1-G93A model of amyotrophic lateral sclerosis

(2012) Renzo Mancuso et al.   Amyotrophic Lateral Sclerosis

The mitochondrial calcium regulator cyclophilin D is an essential component of oestrogen-mediated neuroprotection in amyotrophic lateral sclerosis

(2012) Hyun Jeong Kim et al.   BRAIN

Elevated PGC-1α Activity Sustains Mitochondrial Biogenesis and Muscle Function without Extending Survival in a Mouse Model of Inherited ALS

(2012) Sandrine Da Cruz et al.   Cell Metabolism

Reduced Activity of AMP-Activated Protein Kinase Protects against Genetic Models of Motor Neuron Disease

(2012) M. A. Lim et al.   JOURNAL OF NEUROSCIENCE

Genomic and Proteomic Profiling Reveals Reduced Mitochondrial Function and Disruption of the Neuromuscular Junction Driving Rat Sarcopenia

(2012) C. Ibebunjo et al.   MOLECULAR AND CELLULAR BIOLOGY

Different Human Copper-Zinc Superoxide Dismutase Mutants, SOD1G93A and SOD1H46R, Exert Distinct Harmful Effects on Gross Phenotype in Mice

(2012) Lei Pan et al.   PLoS One

The in vivo contribution of motor neuron TrkB receptors to mutant SOD1 motor neuron disease

(2011) J. Zhai et al.   HUMAN MOLECULAR GENETICS

Sixteen years and counting: an update on leptin in energy balance

(2011) Laurent Gautron et al.   JOURNAL OF CLINICAL INVESTIGATION

Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis

(2011) Laura Ferraiuolo et al.   Nature Reviews Neurology

Metformin Treatment Has No Beneficial Effect in a Dose-Response Survival Study in the SOD1G93A Mouse Model of ALS and Is Harmful in Female Mice

(2011) Hannah M. Kaneb et al.   PLoS One

A decrease in body mass index is associated with faster progression of motor symptoms and shorter survival in ALS

(2010) Ali Jawaid et al.   Amyotrophic Lateral Sclerosis

ALS disease onset may occur later in patients with pre-morbid diabetes mellitus

(2010) A. Jawaid et al.   EUROPEAN JOURNAL OF NEUROLOGY

Prdm16 determines the thermogenic program of subcutaneous white adipose tissue in mice

(2010) Patrick Seale et al.   JOURNAL OF CLINICAL INVESTIGATION

Patients with elevated triglyceride and cholesterol serum levels have a prolonged survival in amyotrophic lateral sclerosis

(2010) J. Dorst et al.   JOURNAL OF NEUROLOGY

Energy metabolism in amyotrophic lateral sclerosis

(2010) Luc Dupuis et al.   LANCET NEUROLOGY

A historical perspective on leptin

(2010) Douglas L Coleman   NATURE MEDICINE

Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism

(2010) P.-M. Chiang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Oxidative stress sensitivity in ALS muscle cells

(2009) Luc Dupuis   EXPERIMENTAL NEUROLOGY

The mitochondrial permeability transition pore in motor neurons: Involvement in the pathobiology of ALS mice

(2009) Lee J. Martin et al.   EXPERIMENTAL NEUROLOGY

Transcriptional control of brown adipocyte development and physiological function--of mice and men

(2009) P. Seale et al.   GENES & DEVELOPMENT

Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

(2009) Hristelina Ilieva et al.   JOURNAL OF CELL BIOLOGY

Hypermetabolism in ALS patients: an early and persistent phenomenon

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

Incidence of amyotrophic lateral sclerosis in Europe

(2009) G. Logroscino et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Muscle Mitochondrial Uncoupling Dismantles Neuromuscular Junction and Triggers Distal Degeneration of Motor Neurons

(2009) Luc Dupuis et al.   PLoS One

High metabolic level in patients with familial amyotrophic lateral sclerosis

(2008) Benoit Funalot et al.   Amyotrophic Lateral Sclerosis

Role of GluR1 in Activity-Dependent Motor System Development

(2008) L. Zhang et al.   JOURNAL OF NEUROSCIENCE

Genetic background determines metabolic phenotypes in the mouse

(2008) Marie-France Champy et al.   MAMMALIAN GENOME

Dyslipidemia is a protective factor in amyotrophic lateral sclerosis

(2008) L. Dupuis et al.   NEUROLOGY

Mitochondrial mechanisms of estrogen neuroprotection

(2007) James W. Simpkins et al.   BRAIN RESEARCH REVIEWS

Effects of estrogen on lifespan and motor functions in female hSOD1 G93A transgenic mice

(2007) Chan-Il Choi et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES