title: A plural role for lipids in motor neuron diseases: energy, signaling and structure.
abstract: Motor neuron diseases (MNDs) are characterized by selective death of motor neurons and include mainly adult-onset amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). Neurodegeneration is not the single pathogenic event occurring during disease progression. There are multiple lines of evidence for the existence of defects in lipid metabolism at peripheral level. For instance, hypermetabolism is well characterized in ALS, and dyslipidemia correlates with better prognosis in patients. Lipid metabolism plays also a role in other MNDs. In SMA, misuse of lipids as energetic nutrients is described in patients and in related animal models. The composition of structural lipids in the central nervous system is modified, with repercussion on membrane fluidity and on cell signaling mediated by bioactive lipids. Here, we review the main epidemiologic and mechanistic findings that link alterations of lipid metabolism and motor neuron degeneration, and we discuss the rationale of targeting these modifications for therapeutic management of MNDs.
authors.affiliation: Mécanismes Centraux et Périphériques de la Neurodégénerescence, INSERM U1118 Strasbourg, France ; UMRS1118, Fédération de Médecine Translationnelle de Strasbourg Université de Strasbourg, France.
authors.forename: Florent
authors.initials: F
authors.lastname: Schmitt
authors.affiliation: Mécanismes Centraux et Périphériques de la Neurodégénerescence, INSERM U1118 Strasbourg, France ; UMRS1118, Fédération de Médecine Translationnelle de Strasbourg Université de Strasbourg, France.
authors.forename: Ghulam
authors.initials: G
authors.lastname: Hussain
authors.affiliation: Mécanismes Centraux et Périphériques de la Neurodégénerescence, INSERM U1118 Strasbourg, France ; UMRS1118, Fédération de Médecine Translationnelle de Strasbourg Université de Strasbourg, France.
authors.forename: Luc
authors.initials: L
authors.lastname: Dupuis
authors.affiliation: Mécanismes Centraux et Périphériques de la Neurodégénerescence, INSERM U1118 Strasbourg, France ; UMRS1118, Fédération de Médecine Translationnelle de Strasbourg Université de Strasbourg, France.
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Loeffler
authors.affiliation: Mécanismes Centraux et Périphériques de la Neurodégénerescence, INSERM U1118 Strasbourg, France ; UMRS1118, Fédération de Médecine Translationnelle de Strasbourg Université de Strasbourg, France.
authors.forename: Alexandre
authors.initials: A
authors.lastname: Henriques
keywords: ALS; SBMA; SMA; lipid; metabolism; motor neuron