title: The metabolic hypothesis in amyotrophic lateral sclerosis: insights from mutant Cu/Zn-superoxide dismutase mice.
abstract: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by selective loss of motor neurons and progressive muscle atrophy. A subset of patients harbors point mutations in the gene encoding Cu/Zn-superoxide dismutase (SOD1), which allowed the generation of transgenic mice that express different SOD1 mutations and develop an ALS-like pathology. Recently, we reported in these mice the occurrence of a characteristic defect in energy homeostasis and the beneficial effect on the course of the disease of a high-energy fat-enriched diet. In this review, we discuss the implication of these findings in the light of classical clinical observations concerning metabolic alterations in human ALS.
authors.affiliation: Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Inserm U-692, Université Louis Pasteur, Faculté de Médecine, 11, rue Humann, 67085 Strasbourg cedex, France.
authors.forename: Jose-Luis
authors.initials: JL
authors.lastname: Gonzalez de Aguilar
authors.forename: Luc
authors.initials: L
authors.lastname: Dupuis
authors.forename: Hugues
authors.initials: H
authors.lastname: Oudart
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Loeffler
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000201:enzymology / Q000378:metabolism*; D000818:Animals; D016923:Cell Death; D004734:Energy Metabolism; D006706:Homeostasis / Q000235:genetics*; D006801:Humans; D051379:Mice; D009046:Motor Neurons / Q000378:metabolism; D013482:Superoxide Dismutase / Q000235:genetics / Q000378:metabolism*
chemical_list: D013482:Superoxide Dismutase