A5031012148- Amyotrophic Lateral Sclerosis Research
- Neurogenetic and Muscular Disorders Research
- Nerve injury and regeneration
- Parkinson's Disease Mechanisms and Treatments
- Muscle Physiology and Disorders
- Neurological diseases and metabolism
- Biochemical Acid Research Studies
- Neurobiology and Insect Physiology Research
- Hypothalamic control of reproductive hormones
- Regulation of Appetite and Obesity
- Adipose Tissue and Metabolism
- Genetic Neurodegenerative Diseases
- Neuropeptides and Animal Physiology
- Alzheimer's disease research and treatments
- Growth Hormone and Insulin-like Growth Factors
- Neuroinflammation and Neurodegeneration Mechanisms
- Prion Diseases and Protein Misfolding
- Melanoma and MAPK Pathways
- Protein Kinase Regulation and GTPase Signaling
- Mitochondrial Function and Pathology
- Cholinesterase and Neurodegenerative Diseases
- Ovarian function and disorders
- Biochemical Analysis and Sensing Techniques
- Neuroendocrine regulation and behavior
- Histone Deacetylase Inhibitors Research
Université de Strasbourg
2013-2024
Inserm
2013-2024
Mécanismes Centraux et Périphériques de la Neurodégénérescence
2011-2022
Institut National de Recherche en Santé Publique
2016
Centre Hospitalier Universitaire de Tours
2008
Assistance Publique – Hôpitaux de Paris
2008
Centre National de la Recherche Scientifique
1999-2004
University of Córdoba
1993-2001
Hospital Municipal de Badalona
2001
Laboratoire de Chimie Organique
2000-2001
Amyotrophic lateral sclerosis (ALS) is the most serious form of degenerative motor neuron disease in adults, characterized by upper and lower degeneration, skeletal muscle atrophy, paralysis, death. High prevalence malnutrition weight loss adversely affect quality life. Moreover, two thirds patients develop a hypermetabolism unknown cause, leading to increased resting energy expenditure. Inasmuch as lipids are major source for muscles, we determined status population with ALS investigated...
Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disease characterized by selective loss of motor neurons and progressive muscle wasting. Growing evidence indicates that mitochondrial dysfunction, not only occurring in but also skeletal muscle, may play a crucial role the pathogenesis. In this regard, life expectancy ALS G93A mouse line extended creatine, intracellular energy shuttle ameliorates function. Moreover, population patients with sporadic exhibits generalized...
Amyotrophic lateral sclerosis (ALS) is characterized by motoneuron (MN) degeneration, generalized weakness, and muscle atrophy. The premature death of MNs thought to be a determinant in the onset this disease. In transgenic mouse model ALS expressing G86R mutant superoxide dismutase 1 (mSOD1), we demonstrated previously that CREB (cAMP response element-binding protein)-binding protein (CBP) histone acetylation levels were specifically decreased nuclei degenerating MNs. We show here oxidative...
Amyotrophic lateral sclerosis (ALS), the most frequent adult onset motor neuron disease, is associated with hypermetabolism linked to defects in muscle mitochondrial energy metabolism such as ATP depletion and increased oxygen consumption. It remains unknown whether abnormalities are causally involved destruction of neuromuscular junction (NMJ) subsequent degeneration during ALS.We studied transgenic mice muscular overexpression uncoupling protein 1 (UCP1), a potent uncoupler, model...
Abstract Nogo, a protein inhibiting axonal regeneration, exhibits characteristic isoform‐specific pattern of expression in skeletal muscle transgenic mice and patients with amyotrophic lateral sclerosis. Here, the increased levels Nogo‐A or Nogo‐B biopsies 15 sclerosis significantly correlated severity clinical disability degree fiber atrophy. immunoreactivity was observed selectively atrophic slow‐twitch type I fibers. These results suggest that Nogo is marker severity. Ann Neurol 2005;57:553–556
Muscle atrophy is a major hallmark of amyotrophic lateral sclerosis (ALS), the most frequent adult-onset motor neuron disease. To define full set alterations in gene expression skeletal muscle during course disease, we used G86R superoxide dismutase-1 transgenic mouse model ALS and performed high-density oligonucleotide microarrays. We compared these data to those obtained by axotomy-induced denervation. A regulations muscles resembled surgically denervated muscles, but many others appeared...
Amyotrophic lateral sclerosis (ALS) is the most common adult motor neuron disease, causing degeneration, muscle atrophy, paralysis, and death. Despite this degenerative process, a stable hypermetabolic state has been observed in large subset of patients. Mice expressing mutant form Cu/Zn-superoxide dismutase (mSOD1 mice) constitute an animal model ALS that, like patients, exhibits unexpectedly increased energy expenditure. Counterbalancing for increase with high-fat diet extends lifespan...
Amyotrophic lateral sclerosis (ALS) is a fatal adult-onset disease characterized by upper and lower motor neuron degeneration, muscle wasting paralysis. Growing evidence suggests link between changes in lipid metabolism ALS. Here, we used UPLC/TOF-MS to survey the lipidome SOD1(G86R) mice, model of Significant expression were evident spinal cord skeletal before overt neuropathology. In silico analysis also revealed appreciable sphingolipids including ceramides glucosylceramides (GlcCer)....
Objective Data from mouse models of amyotrophic lateral sclerosis (ALS) suggest early morphological changes in neuromuscular junctions (NMJs), with loss nerve–muscle contact. Overexpression the neurite outgrowth inhibitor Nogo-A muscle may play a role this endplate innervation. Methods We used confocal and electron microscopy to study structure NMJs samples collected nine ALS patients (five early-stage four long-term survivors). correlated results clinical electrophysiological data,...
A proportion of patients with pure lower motor neuron syndrome (LMNS) progress to amyotrophic lateral sclerosis (ALS). Early detection this progression is impossible, which delays the patient's access treatment. Muscle expression Nogo-A a new candidate marker ALS. We tested whether in muscle biopsy from LMNS predicts ALS.Thirty-three who had undergone during diagnostic workup spinal were observed for 12 months. was measured by Western blot samples and compared final diagnosis.Nogo-A detected...
Spasticity is a common and disabling symptom observed in patients with central nervous system diseases, including amyotrophic lateral sclerosis, disease affecting both upper lower motor neurons. In spasticity traditionally thought to be the result of degeneration neurons cerebral cortex, although other neuronal types, particular serotonergic neurons, might also represent cause spasticity. We performed pathology study seven sclerosis six control subjects that suffer from degenerative process...