Net sulfatide synthesis, galactosylceramide sulfotransferase and arylsulfatase a activity in the developing cerebrum and cerebellum of normal mice and myelin-deficient jimpy mice

Myelin

“…1), cerebrosides are present at more significant concentratiolls. Net sulfatide synthesis was found to be preserved during the first phase (9-12 days) of jp brain development, but was lost later during active myelination (Burkart et al, 1981). This synthesis of cerebrosides and sulfatides early in brain development was not observed by Yahara et al (1981), employing a different method of assay utilizing high-pressure liquid chromatography, which showed lower normal tissue levels than the previous studies.…”

“…It is dear that the striking morphologieal and biochemieal changes in the jimpy mutant are brain-specific allel are selective for the oligodt'ndrocyte. The morphological evidence of oligodendroeyte necJ'(Jsis (C. Meier ,md Bischoff,19H) slipported by ceIl-kinetic analysis (Skoff, 1982), the deficiencies that occur simultaneously in many and varied myelin constituents with indication of faIling tissue levels and biosynthetic activities Kandutsch and Saucier, 1972;Burkart et al, 1981) during development, and the lethaI effect on the animal are consistent with a shortened life-span of the oligodendrocyte in this mutant. The coordinated involvement of several myelin components would be compatible with adeletion of several structural genes, perhaps in contiguous position, and functionallinkage of the chromosome.…”

“…The Km of the last was identical in control and mutant CNS. A marked reduction in jimpy brain has been reported for sulfotransferases as weil: 3-phosphoadenosine 5'phosphosulfate (PAPS)-galactocerebroside sulfotransferase Herschkowitz et al, 1971;Burkart et al, 1981) and PAPS-psychosine sulfotransferase (Nussbaum and Mandel, 1972). There is no evidence of an altered structure of the cerebroside sulfotransferase in the jimpy according to Michaelis constant (Km), pH optimum, and heat stability, which do not differ from control (Matthieu et al, 1972).…”

“…in contlol ami jimpy brain dnfing dcvdopmclll (Bowen ane! Radin, 1969;Deshmukh et al, 1971;Burkart et al, 1981). In the jimpy mutant, however, 47% mOle cerebrosie!e galactosidase could be dispen,ee!…”

“…Arylsulfatase A activity is not changee! in the brain of the jimpy mutant (Sarlicvc et al, 1978;Burkart et al, 1981). There is no ine!ication of increasee!…”