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The Journal of Neuroscience, July 15, 2000, 20(14):5225-5233

Early Onset of Axonal Degeneration in Double (plp/mag/) and Hypomyelinosis in Triple (plp/mbp/mag/) Mutant Mice

Thomas Uschkureit¹, Olaf Spörkel¹, Jens Stracke¹, Heinrich Büssow², and Wilhelm Stoffel¹

¹ Laboratory for Molecular Neuroscience, University of Cologne, D-50931 Cologne, Germany, and ² Institute of Anatomy, University of Bonn, D-53115 Bonn, Germany

Double (plp/mag/) and triple (plp/mbp/mag/) null-allelic mouse lines deficient in proteolipid protein (PLP), myelin-associated glycoprotein (MAG), and myelin basic protein (MBP) were generated and characterized genetically, biochemically, and morphologically including their behavioral capacities. The plp/mag/ mutant develops a rapidly progressing axon degeneration in CNS with severe cognitive and motor coordinative deficits but has a normal longevity. CNS axons of the plp/mbp/mag/ mouse are hypomyelinated and ensheathed by "pseudomyelin" with disturbed protein and complex lipid composition. The shiverer trait in the plp/mbp/mag/ similar to the plp/mbp/ mutant is significantly ameliorated, and its lifespan is considerably prolonged. The longevity of these dysmyelinosis mouse mutants recommends them as suitable models for the long-term evaluation of stem cell therapeutic strategies.

Key words: double mutants; triple mutant; myelin proteins; myelin lipids; ultrastructure of myelin; behavioral tests

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Copyright © 2000 Society for Neuroscience  0270-6474/00/20145225-09$05.00/0

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