Abstract
Two glycosyl transferases involved in the biosynthesis of glycolipids were investigated in the brain of myelin deficient mutants, Jimpy and Quaking mice. The first, UDP-galactose-ceramide galactosyl transferase, which catalyzes the synthesis of cerebroside from UDP-galactose and ceramide, was markedly reduced in Jimpy mouse and its activity did not change during the myelination period. In Quaking mouse the activity of this enzyme was also reduced but showed characteristic developmental changes, similar to those observed in normal mouse brain. The second glycosyl transferase, UDP-glucose-ceramide glucosyl transferase, involved in the biosynthesis of gangliosides, had a different pattern of developmental changes, and normal levels of activity were found in mutant brain. These data, as well as others discussed in the paper, indicate the existence in the postnatal brain development of two different glycosyl transferase systems, presumably with independent genetic controls.
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