title: Role of the N-methyl-d-aspartate receptors complex in amyotrophic lateral sclerosis.
abstract: Amyotrophic lateral sclerosis (ALS) is an adult onset neurodegenerative disease pathologically characterized by the massive loss of motor neurons in the spinal cord, brain stem and cerebral cortex. There is a consensus in the field that ALS is a multifactorial pathology and a number of possible mechanisms have been suggested. Among the proposed hypothesis, glutamate toxicity has been one of the most investigated. Alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor mediated cell death and impairment of the glutamate-transport system have been suggested to play a central role in the glutamate-mediated motor neuron degeneration. In this context, the role played by the N-methyl-d-aspartate (NMDA) receptor has received considerable less attention notwithstanding its high Ca(2+) permeability, expression in motor neurons and its importance in excitotoxicity. This review overviews the critical role of NMDA-mediated toxicity in ALS, with a particular emphasis on the endogenous modulators of the NMDAR.
authors.affiliation: Molecular Neurobiology Unit, Experimental Neurology, Fondazione Santa Lucia, Rome Italy.
authors.forename: Alida
authors.initials: A
authors.lastname: Spalloni
authors.forename: Michele
authors.initials: M
authors.lastname: Nutini
authors.forename: Patrizia
authors.initials: P
authors.lastname: Longone
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism* / Q000473:pathology; D016923:Cell Death; D006801:Humans; D009046:Motor Neurons / Q000473:pathology; D016194:Receptors, N-Methyl-D-Aspartate / Q000378:metabolism / Q000502:physiology*; D013116:Spinal Cord / Q000378:metabolism
chemical_list: D016194:Receptors, N-Methyl-D-Aspartate