Accumulation of galactosylsphingosine (psychosine) does not interfere with phosphorylation and methylation of myelin basic protein in the twitcher mouse
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In attempts to elucidate mechanisms of demyelination in the twitcher mouse (Twi), phosphorylation and methylation of myelin basic protein (MBP) were examined in the brainstem and spinal cord of this species. Phosphorylation of MBP in isolated myelin by an endogenous kinase and an exogenous [32P]ATP was not impaired and protein kinase C activity in the brain cytosol was not reduced. When the methylation of an arginine residue of MBP was examined in slices of the brainstem and spinal cord, using [3H]methionine as a donor of the methyl groups, no difference was found between Twi and the controls. Radioactivity of the [3H] methionine residue of MBP of Twi was also similar to that of the controls. Thus, accumulation of psychosine in Twi does not interfere with the activity of endogenous kinase, methylation of MBP, and the synthesis and transport of MBP into myelin membrane.
Key WordsTwitcher mouse myelin basic protein psychosine phosphorylation methylation
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- 1.Suzuki, K., and Suzuki, Y. 1983. Galactosylceramide lipidosis: globoid cell leukodystrophy (Krabbe's disease). Pages 857–880,in Stanbury, J. B., Wyngaarden, J. B., Fredrickson, D. S., Goldstein, J. L., and Brown, M. S. (eds.), The Metabolic Basis of-Inherited Disease. McGraw-Hill, New York.Google Scholar
- 3.Miyatake, T., and Suzuki, K. 1972. Globoidcell leukodystdrophy: additional deficiency of psychosine galactosidase. Biochem. Biophys. Res. Commun. 48:538–543.Google Scholar
- 29.Takahashi, H., and Suzuki, K. 1984. Demyelination in the spinal cord of murine globoid cell leukodystrophy (the twitcher mouse). Acta Neuropathol. (Berl.) 62:298–308.Google Scholar
- 31.Stoner, G. L. 1983. Proposed role for phosporylation in the folding of myelin basic protein. Trans. Am. Soc. Neurochem. 14: 164 (Abstr).Google Scholar
- 41.Norton, W. T., and Cammer, W. 1984. Isolation and characterization of myelin. Pages 147–195,in Morell, P. (ed.), Myelin, Plenum Press, New York.Google Scholar