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Evidence on hypomyelination of central nervous system in murine muscular dystrophy

descriptionPublicationkeyboard_double_arrow_right Article 01 May 1985 English Publisher:Elsevier BVJournal:Journal of the Neurological Sciences, volume 68, pages 175-184 (issn: 0022-510X,

Authors: Shigekatsu Tsuji; Hiroshi Matsushita;

doi: 10.1016/0022-510x(85)90098-x

pmid: 2989438

Evidence on hypomyelination of central nervous system in murine muscular dystrophy

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Abstract

To elucidate the disturbances of myelin metabolism in the nerve tissue of murine muscular dystrophy, the lipid composition of and the developmental changes in 2', 3'-cyclic nucleotide 3'-phosphohydrolase (CNPase) and cholesterol ester hydrolase (CEHase) activities in the purified CNS myelin of dystrophic mice were determined. Several kinds of lipids, total galactolipid and cerebroside sulfatide levels were significantly reduced as compared with controls. Total cholesterol levels in the spinal cord of dystrophic mice were moderately higher. CEHase and, to a lesser degree, CNPase activities were reduced in the purified myelin of the CNS of the dystrophic mice. The reduced myelin CEHase activity in dystrophic mice suggests that impairment of hydrolysis of steryl esters may be important in the development of hypomyelination of the CNS.

Keywords

Central Nervous System, Phosphoric Diester Hydrolases, Muscular Dystrophy, Animal, Sterol Esterase, Lipids, Mice, Inbred C57BL, Mice, Microscopy, Electron, 2',3'-Cyclic Nucleotide 3'-Phosphodiesterase, Animals, 2',3'-Cyclic-Nucleotide Phosphodiesterases, Carboxylic Ester Hydrolases, Myelin Sheath

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