title: Death Receptors in the Selective Degeneration of Motoneurons in Amyotrophic Lateral Sclerosis.
abstract: While studies on death receptors have long been restricted to immune cells, the last decade has provided a strong body of evidence for their implication in neuronal death and hence neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS). ALS is a fatal paralytic disorder that primarily affects motoneurons in the brain and spinal cord. A neuroinflammatory process, associated with astrocyte and microglial activation as well as infiltration of immune cells, accompanies motoneuron degeneration and supports the contribution of non-cell-autonomous mechanisms in the disease. Hallmarks of Fas, TNFR, LT-βR, and p75(NTR) signaling have been observed in both animal models and ALS patients. This review summarizes to date knowledge of the role of death receptors in ALS and the link existing between the selective loss of motoneurons and neuroinflammation. It further suggests how this recent evidence could be included in an ultimate multiapproach to treat patients.
authors: Inserm-Avenir team, The Mediterranean Institute of Neurobiology (INMED), 13288 Marseille, France ; Neurodegenerative Studies Laboratory, Brain Mind Institute, The Swiss Federal Institute of Technology Lausanne (EPFL), CH-1015 Lausanne, Switzerland.
authors: Julianne
authors: J
authors: Aebischer
authors: Inserm-Avenir team, The Mediterranean Institute of Neurobiology (INMED), 13288 Marseille, France ; Department of Medical Genetics, University of Lausanne, CH-1005 Lausanne, Switzerland.
authors: Nathalie
authors: N
authors: Bernard-Marissal
authors: Inserm-Avenir team, The Mediterranean Institute of Neurobiology (INMED), 13288 Marseille, France ; The Mediterranean Institute of Neurobiology (INMED), Inserm UMR901, 13288 Marseille, France.
authors: Brigitte
authors: 0000-0003-2059-5438
authors: B
authors: Pettmann
authors: Inserm-Avenir team, The Mediterranean Institute of Neurobiology (INMED), 13288 Marseille, France ; The Neuroscience Institute Montpellier (INM), INSERM UMR1051, Saint Eloi Hospital, 34091 Montpellier, France.
authors: Cédric
authors: C
authors: Raoul