title: Links between electrophysiological and molecular pathology of amyotrophic lateral sclerosis.
abstract: Multiple deficits have been described in amyotrophic lateral sclerosis (ALS), from the first changes in normal functioning of the motoneurons and glia to the eventual loss of spinal and cortical motoneurons. In this review, current results, including changes in size, and electrical properties of motoneurons, glutamate excitotoxicity, calcium buffering, deficits in mitochondrial and cellular transport, impediments to proteostasis which lead to stress of the endoplasmic reticulum (ER), and glial contributions to motoneuronal vulnerability are recapitulated. Results are mainly drawn from the mutant SOD1 mouse model of ALS, and emphasis is placed on early changes that precede the onset of symptoms and the interplay between molecular and electrical processes.
authors: Department of Physiology, Northwestern University, Feinberg School of Medicine, Chicago, IL 60611, USA. katharina-quinlan@northwestern.edu
authors: Katharina A
authors: KA
authors: Quinlan
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000201:enzymology / Q000235:genetics / Q000503:physiopathology*; D000818:Animals; D001253:Astrocytes / Q000378:metabolism / Q000502:physiology; D002118:Calcium / Q000378:metabolism; D002135:Calcium-Binding Proteins / Q000378:metabolism; D004195:Disease Models, Animal; D055724:Electrophysiological Phenomena; D004721:Endoplasmic Reticulum / Q000502:physiology; D018698:Glutamic Acid / Q000378:metabolism; D053078:Membrane Potential, Mitochondrial; D051379:Mice; D009046:Motor Neurons / Q000502:physiology; D009431:Neural Conduction; D059748:Proteolysis; D013312:Stress, Physiological; D013482:Superoxide Dismutase / Q000235:genetics / Q000378:metabolism; D000072105:Superoxide Dismutase-1
chemical_list: D002135:Calcium-Binding Proteins; D018698:Glutamic Acid; C000606291:Sod1 protein, mouse; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1; D002118:Calcium