title: Myelin composition of spinal cord in a model of amyotrophic lateral sclerosis (ALS) in SOD1G93A transgenic rats.
abstract: We present the results of biochemical and electron microscopic (EM) examinations of the spinal cord myelin from SOD1G93A transgenic Sprague Dawley rats in the early and late symptom-free period of the disease (60 and 93 days of life) and after four-leg paralysis has occurred (120 days of life). Biochemical and ultrastructural changes of myelin started already in the symptom-free period and become most pronounced in the paralyzed animals. Biochemical examinations indicated a decrease of lipids, phospholipids, cholesterol and cerebrosides. The pattern of particular phospholipids was in the normal range. A progressive decrease of the percentages of proteolipid, DM-20 and Wolfgram proteins was evident. Myelin basic proteins I and II were less affected. In EM,massive myelin disorganization was observed.
authors.affiliation: Neuromuscular Unit Medical Research Centre, Polish Academy of Sciences, 5 Pawiñskiego St., 02-108 Warsaw, Poland. neurmyol@cmdik.pan.pl
authors.forename: Irena
authors.initials: I
authors.lastname: Niebroj-Dobosz
authors.forename: Janina
authors.initials: J
authors.lastname: Rafałowska
authors.forename: Anna
authors.initials: A
authors.lastname: Fidziańska
authors.forename: Roman
authors.initials: R
authors.lastname: Gadamski
authors.forename: Paweł
authors.initials: P
authors.lastname: Grieb
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology*; D000818:Animals; D030801:Animals, Genetically Modified; D004195:Disease Models, Animal; D009186:Myelin Sheath / Q000737:chemistry* / Q000648:ultrastructure*; D051381:Rats; D017207:Rats, Sprague-Dawley; D013116:Spinal Cord / Q000737:chemistry / Q000648:ultrastructure*; D013482:Superoxide Dismutase / Q000235:genetics
chemical_list: C109415:SOD1 G93A protein; D013482:Superoxide Dismutase