Evidence on hypomyelination of central nervous system in murine muscular dystrophy

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.