Luc Dupuis

Summary

Publications

  1. doi request reprint Energy metabolism in amyotrophic lateral sclerosis
    Luc Dupuis
    INSERM U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Strasbourg, France
    Lancet Neurol 10:75-82. 2011
  2. doi request reprint Mitochondrial quality control in neurodegenerative diseases
    Luc Dupuis
    INSERM, U1118, Strasbourg F 67085, France Université de Strasbourg, Fédération de Médecine Translationnelle FMTS, UMRS1118, Strasbourg F 67085, France Electronic address
    Biochimie 100:177-83. 2014
  3. pmc Fumaric acid esters stimulate astrocytic VEGF expression through HIF-1α and Nrf2
    Diana Wiesner
    Department of Neurology, University of Ulm, Ulm, Germany
    PLoS ONE 8:e76670. 2013
  4. pmc G-CSF protects motoneurons against axotomy-induced apoptotic death in neonatal mice
    Alexandre Henriques
    Sygnis Bioscience, Im Neuenheimer Feld 515, 69120 Heidelberg, Germany
    BMC Neurosci 11:25. 2010
  5. ncbi request reprint Skeletal muscle in motor neuron diseases: therapeutic target and delivery route for potential treatments
    Luc Dupuis
    INSERM, U692, Strasbourg, F 67085 France
    Curr Drug Targets 11:1250-61. 2010
  6. pmc Muscle mitochondrial uncoupling dismantles neuromuscular junction and triggers distal degeneration of motor neurons
    Luc Dupuis
    INSERM, U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Strasbourg, France
    PLoS ONE 4:e5390. 2009
  7. pmc Platelet serotonin level predicts survival in amyotrophic lateral sclerosis
    Luc Dupuis
    INSERM U692, Strasbourg, France
    PLoS ONE 5:e13346. 2010
  8. ncbi request reprint Up-regulation of mitochondrial uncoupling protein 3 reveals an early muscular metabolic defect in amyotrophic lateral sclerosis
    Luc Dupuis
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, EA 3433, Universite Louis Pasteur, Faculte de Medecine, 11 rue Humann, 67085 Strasbourg Cedex, France
    FASEB J 17:2091-3. 2003
  9. ncbi request reprint Gene profiling of skeletal muscle in an amyotrophic lateral sclerosis mouse model
    Jose Luis Gonzalez de Aguilar
    Institut National de la Sante et de la Recherche Medicale, U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Strasbourg, France
    Physiol Genomics 32:207-18. 2008
  10. ncbi request reprint Mitochondria in amyotrophic lateral sclerosis: a trigger and a target
    Luc Dupuis
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, U692 INSERM, Faculte de Medecine, Universite Louis Pasteur, Strasbourg, France
    Neurodegener Dis 1:245-54. 2004

Collaborators

Detail Information

Publications37

  1. doi request reprint Energy metabolism in amyotrophic lateral sclerosis
    Luc Dupuis
    INSERM U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Strasbourg, France
    Lancet Neurol 10:75-82. 2011
    ..Altogether, these results have clinical consequences for the management of defective energy metabolism in patients with ALS and pave the way for future therapeutic interventions...
  2. doi request reprint Mitochondrial quality control in neurodegenerative diseases
    Luc Dupuis
    INSERM, U1118, Strasbourg F 67085, France Université de Strasbourg, Fédération de Médecine Translationnelle FMTS, UMRS1118, Strasbourg F 67085, France Electronic address
    Biochimie 100:177-83. 2014
    ..This review provides an update on the most recent literature on mitochondrial quality control and its impairment during neurodegenerative diseases. ..
  3. pmc Fumaric acid esters stimulate astrocytic VEGF expression through HIF-1α and Nrf2
    Diana Wiesner
    Department of Neurology, University of Ulm, Ulm, Germany
    PLoS ONE 8:e76670. 2013
    ..These studies show that FAEs elicit different signaling pathways in cell types from the central nervous system, in particular a pseudo-hypoxic response in astrocytes. Disease relevant mutations might affect this response. ..
  4. pmc G-CSF protects motoneurons against axotomy-induced apoptotic death in neonatal mice
    Alexandre Henriques
    Sygnis Bioscience, Im Neuenheimer Feld 515, 69120 Heidelberg, Germany
    BMC Neurosci 11:25. 2010
    ..Here we studied whether G-CSF is able to protect motoneurons from purely apoptotic cell death induced by a monocausal paradigm, neonatal sciatic nerve axotomy...
  5. ncbi request reprint Skeletal muscle in motor neuron diseases: therapeutic target and delivery route for potential treatments
    Luc Dupuis
    INSERM, U692, Strasbourg, F 67085 France
    Curr Drug Targets 11:1250-61. 2010
    ..Apart from its potential pathogenic role, skeletal muscle pathophysiological events might be a target for treatments and/or be a preferential route for targeting motor neurons...
  6. pmc Muscle mitochondrial uncoupling dismantles neuromuscular junction and triggers distal degeneration of motor neurons
    Luc Dupuis
    INSERM, U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Strasbourg, France
    PLoS ONE 4:e5390. 2009
    ..It remains unknown whether muscle abnormalities in energy metabolism are causally involved in the destruction of neuromuscular junction (NMJ) and subsequent motor neuron degeneration during ALS...
  7. pmc Platelet serotonin level predicts survival in amyotrophic lateral sclerosis
    Luc Dupuis
    INSERM U692, Strasbourg, France
    PLoS ONE 5:e13346. 2010
    ..Serotonin is involved in a range of functions altered in ALS, including motor neuron excitability and energy metabolism. However, whether serotoninergic activity represents a disease modifier of ALS natural history remains unknown...
  8. ncbi request reprint Up-regulation of mitochondrial uncoupling protein 3 reveals an early muscular metabolic defect in amyotrophic lateral sclerosis
    Luc Dupuis
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, EA 3433, Universite Louis Pasteur, Faculte de Medecine, 11 rue Humann, 67085 Strasbourg Cedex, France
    FASEB J 17:2091-3. 2003
    ..Moreover, since skeletal muscle is a key metabolic tissue, our findings suggest that ALS may not solely arise from neuronal events but also from more generalized metabolic defects...
  9. ncbi request reprint Gene profiling of skeletal muscle in an amyotrophic lateral sclerosis mouse model
    Jose Luis Gonzalez de Aguilar
    Institut National de la Sante et de la Recherche Medicale, U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Strasbourg, France
    Physiol Genomics 32:207-18. 2008
    ..Second, they identify specific gene regulations to be explored in the search for therapeutic strategies that could alleviate disease before motor neuron death manifests clinically...
  10. ncbi request reprint Mitochondria in amyotrophic lateral sclerosis: a trigger and a target
    Luc Dupuis
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, U692 INSERM, Faculte de Medecine, Universite Louis Pasteur, Strasbourg, France
    Neurodegener Dis 1:245-54. 2004
    ..In this review, we will focus on the mitochondrial dysfunction linked to ALS and the cause-and-effect relationships between mitochondria and the pathological mechanisms thought to be involved in the disease...
  11. doi request reprint Mice with a mutation in the dynein heavy chain 1 gene display sensory neuropathy but lack motor neuron disease
    Luc Dupuis
    INSERM, U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Strasbourg, F 67085, France
    Exp Neurol 215:146-52. 2009
    ..Altogether, these findings show that dynein heavy chain mutation triggers sensory neuropathy rather than motor neuron disease...
  12. pmc Increased peripheral lipid clearance in an animal model of amyotrophic lateral sclerosis
    Anissa Fergani
    Institut National de la Sante et de la Recherche Medicale, U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Strasbourg, F 67085 France
    J Lipid Res 48:1571-80. 2007
    ..Together, our findings show that energy metabolism in mSOD1 mice shifts toward an increase in the peripheral use of lipids. This metabolic shift probably accounts for the protective effect of dietary lipids in this model...
  13. pmc Nogo receptor antagonizes p75NTR-dependent motor neuron death
    Luc Dupuis
    Institut National de la Sante et de la Recherche Medicale, U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Strasbourg, F 67085 France
    Proc Natl Acad Sci U S A 105:740-5. 2008
    ..These findings demonstrate an as-yet-unknown function of NgR in maintaining neuronal survival that may be relevant for motor neuron development and degeneration...
  14. pmc Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in a transgenic mouse model
    Luc Dupuis
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Faculte de Medecine, EA3433, Universite Louis Pasteur, 11 rue Humann, 67085 Strasbourg Cedex, France
    Proc Natl Acad Sci U S A 101:11159-64. 2004
    ..In conclusion, we suggest that hypermetabolism, mainly of muscular origin, may represent by itself an additional driven force involved in increasing motor neuron vulnerability...
  15. ncbi request reprint Early activation of antioxidant mechanisms in muscle of mutant Cu/Zn-superoxide dismutase-linked amyotrophic lateral sclerosis mice
    Natasa Jokic
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Universite Louis Pasteur, Faculte de Medecine, 11 rue Humann, 67085 Strasbourg, France
    Ann N Y Acad Sci 1010:552-6. 2003
    ..Our results are strongly indicative of the early and long-lasting activation of a series of molecular effectors thought to act coordinately in preventing the increased oxidative stress characteristic of ALS...
  16. pmc The neurite outgrowth inhibitor Nogo-A promotes denervation in an amyotrophic lateral sclerosis model
    Natasa Jokic
    INSERM, U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, 11 rue Humann, F 67085 Strasbourg, France
    EMBO Rep 7:1162-7. 2006
    ..This suggests that the expression of Nogo-A occurring early in ALS skeletal muscle could cause repulsion and destabilization of the motor nerve terminals, and subsequent dying back of the axons and motor neurons...
  17. doi request reprint Mutations in cytoplasmic dynein lead to a Huntington's disease-like defect in energy metabolism of brown and white adipose tissues
    Judith Eschbach
    INSERM, U692, Strasbourg, F 67085 France
    Biochim Biophys Acta 1812:59-69. 2011
    ..This peripheral phenotype of dynein mutant mice is similar to that observed in various animal models of HD, lending further support for a functional link between huntingtin and dynein...
  18. doi request reprint [Amyotrophic lateral sclerosis: role of energy deficiency in neuromuscular junction dismantlement]
    Luc Dupuis
    INSERM U692, Laboratoire SMN, Universite Louis Pasteur, UMRS 692, Faculte de Medecine, 11, rue Humann, 67085 Strasbourg, France
    Med Sci (Paris) 24:1077-82. 2008
    ..These results, along with a comparative analysis between the phenotype of mSOD1 mice and ALS patients, suggest new therapeutic strategies and show the interests but also the limits of the animal models...
  19. ncbi request reprint Reticulons as markers of neurological diseases: focus on amyotrophic lateral sclerosis
    Anissa Fergani
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, INSERM U 692, Universite Louis Pasteur, Strasbourg, France
    Neurodegener Dis 2:185-94. 2005
    ..The rearrangement of RTN expression is regulated not only in suffering skeletal muscle but also preceding the onset of symptoms, and may relate to the disease process...
  20. ncbi request reprint Amyotrophic lateral sclerosis: all roads lead to Rome
    Jose Luis Gonzalez de Aguilar
    INSERM, U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Universite Louis Pasteur, Faculte de Medecine, UMRS692, Strasbourg, France
    J Neurochem 101:1153-60. 2007
    ..Identifying different disease subtypes is an unavoidable step toward the understanding of the physiopathology of ALS and will hopefully help to design specific treatments for each subset of patients...
  21. pmc Dynein mutations associated with hereditary motor neuropathies impair mitochondrial morphology and function with age
    Judith Eschbach
    Inserm U1118, Strasbourg F 67085, France
    Neurobiol Dis 58:220-30. 2013
    ..Our results show that dynein function is required for the maintenance of mitochondrial morphology and function with aging and suggest that mitochondrial dysfunction contributes to dynein-dependent neurological diseases, such as SMA-LED...
  22. pmc Tissue specificity and regulation of the N-terminal diversity of reticulon 3
    Franck Di Scala
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, EA 3433, Universite Louis Pasteur, Faculte de Medecine, 11 rue Humann, 67085 Strasbourg Cedex, France
    Biochem J 385:125-34. 2005
    ..These findings suggest that RTN3 isoforms may contribute, by as yet unknown mechanisms, to neuronal survival and plasticity...
  23. ncbi request reprint Nogo provides a molecular marker for diagnosis of amyotrophic lateral sclerosis
    Luc Dupuis
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, EA 3433, Faculte de Medecine, Universite Louis Pasteur, 11 rue Humann, 67085 Strasbourg Cedex, France
    Neurobiol Dis 10:358-65. 2002
    ..Thus, the alteration in Nogo expression pattern, common to sporadic and familial ALS, represents a potential diagnosis tool and points strongly to Nogo having a central role in disease...
  24. ncbi request reprint Nogo expression in muscle correlates with amyotrophic lateral sclerosis severity
    Natasa Jokic
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Universite Louis Pasteur, Strasbourg, France
    Ann Neurol 57:553-6. 2005
    ..Nogo-A immunoreactivity was observed selectively in atrophic slow-twitch type I fibers. These results suggest that Nogo expression in muscle is a marker of amyotrophic lateral sclerosis severity...
  25. ncbi request reprint Sodium valproate exerts neuroprotective effects in vivo through CREB-binding protein-dependent mechanisms but does not improve survival in an amyotrophic lateral sclerosis mouse model
    Caroline Rouaux
    Institut National de la Sante et de la Recherche Medicale, U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Strasbourg F 67085, France
    J Neurosci 27:5535-45. 2007
    ....
  26. ncbi request reprint The metabolic hypothesis in amyotrophic lateral sclerosis: insights from mutant Cu/Zn-superoxide dismutase mice
    Jose Luis Gonzalez de Aguilar
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, INSERM U 692, Universite Louis Pasteur, Faculte de Medecine, 11, rue Humann, 67085 Strasbourg Cedex, France
    Biomed Pharmacother 59:190-6. 2005
    ..In this review, we discuss the implication of these findings in the light of classical clinical observations concerning metabolic alterations in human ALS...
  27. ncbi request reprint Denervation is not a primary cause of prion protein down-regulation occurring in the spinal cord of a transgenic model of amyotrophic lateral sclerosis
    Luc Dupuis
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Universite Louis Pasteur, Faculte de Medecine, 67085 Strasbourg, France
    Ann N Y Acad Sci 973:116-9. 2002
  28. doi request reprint Neuromuscular junction destruction during amyotrophic lateral sclerosis: insights from transgenic models
    Luc Dupuis
    INSERM, U692, Laboratoire SMN, Strasbourg, F 67085, France
    Curr Opin Pharmacol 9:341-6. 2009
    ..Current research should now focus on understanding the relationships between these pathological hallmarks and how such global defects lead to the ALS-linked selective loss of motor neurons...
  29. pmc A mutation in the dynein heavy chain gene compensates for energy deficit of mutant SOD1 mice and increases potentially neuroprotective IGF-1
    Anissa Fergani
    INSERM U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Strasbourg, F 67085 France
    Mol Neurodegener 6:26. 2011
    ..abstract:..
  30. pmc Systemic down-regulation of delta-9 desaturase promotes muscle oxidative metabolism and accelerates muscle function recovery following nerve injury
    Ghulam Hussain
    INSERM, U1118, Mécanismes Centraux et Péripheriques de la Neurodégénérescence, Strasbourg, France
    PLoS ONE 8:e64525. 2013
    ..Overall, these findings provide evidence for a new role of SCD1 in modulating the restorative potential of skeletal muscles...
  31. ncbi request reprint Neuroendocrinology of neurodegenerative diseases. Insights from transgenic mouse models
    Jose Luis Gonzalez de Aguilar
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, EA 3433, Universite Louis Pasteur, Strasbourg, France
    Neuroendocrinology 78:244-52. 2003
    ..Special attention is focused on the contribution of disease transgenic models to elucidate such alterations...
  32. ncbi request reprint Degeneration of serotonergic neurons in amyotrophic lateral sclerosis: a link to spasticity
    Christel Dentel
    U692, INSERM, 67085 Strasbourg, France
    Brain 136:483-93. 2013
    ..Further research is needed to determine whether inverse agonists of 5-hydroxytryptamine-2b/c receptors could be of interest in treating spasticity in patients with amyotrophic lateral sclerosis...
  33. ncbi request reprint Loss of prion protein in a transgenic model of amyotrophic lateral sclerosis
    Luc Dupuis
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, EA 3433 Faculté de Médecine, Universite Louis Pasteur, Strasbourg, France
    Mol Cell Neurosci 19:216-24. 2002
    ..PrP(C) has been shown to play a role in the protection against oxidative stress, and we therefore propose that its down-regulation may contribute at least in part to ALS pathogenesis...
  34. ncbi request reprint Antibody-bound beta-amyloid precursor protein stimulates the production of tumor necrosis factor-alpha and monocyte chemoattractant protein-1 by cortical neurons
    Corinne Mbebi
    Laboratoire de Signalisations Moléculaires et Neurodégénérescence, INSERM, U692, Universite Louis Pasteur, Faculte de Medecine, 11, rue Humann, 67085 Strasbourg Cedex, France
    Neurobiol Dis 19:129-41. 2005
    ..Taken together, our findings provide evidence for a novel mechanism whereby neuronal APP in its full-length configuration induces neuronal death. Such a mechanism might be relevant to neuroinflammatory processes as those observed in AD...
  35. ncbi request reprint [ALS treatment: state of the art in 2014 and perspectives]
    Pierre Francois Pradat
    AP HP, Hopital de la Pitie Salpetriere, Département des Maladies du Système Nerveux, 75013 Paris, France Sorbonne Universités, UPMC Univ Paris 06, INSERM, CNRS, Laboratoire d Imagerie Biomédicale, 75005 Paris, France Electronic address
    Presse Med 43:595-602. 2014
    ..Recent progress in the genetic of ALS may lead to new therapeutic strategies. Results of therapeutic trials with drugs or nutritional intervention are expected in 2014. ..
  36. doi request reprint Cytoplasmic dynein in neurodegeneration
    Judith Eschbach
    INSERM U692, Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Strasbourg, F 67085, France
    Pharmacol Ther 130:348-63. 2011
    ..Here, we critically review the evidence of dynein involvement in different neurodegenerative diseases and discuss potential underlying mechanisms...
  37. ncbi request reprint BBS-induced ciliary defect enhances adipogenesis, causing paradoxical higher-insulin sensitivity, glucose usage, and decreased inflammatory response
    Vincent Marion
    Laboratoire de Physiopathologie des syndromes rares héréditaires, Avenir INSERM, EA3949, Universite de Strasbourg, 11 rue Humann, 67085 Strasbourg, France
    Cell Metab 16:363-77. 2012
    ..Despite increased obesity, glucose tolerance was increased with specific enhanced insulin sensitivity in the fat. This correlated with an active recruitment of MSCs resulting in adipose tissue hyperplasia and decreased in inflammation...