Brain sulfatide and non-lipid sulfate metabolism in leucodystrophic (jimpy) mice

In leucodystrophic (jimpy mutant) mice there is a marked depletion of brain sulfatide as compared to normal ilttermates. Subcellular fractionation showed decreased sulfatide concentrations in microsomal and synaptosomal fractions prepared from jimpy brains. The in vivo incorporation of 35SO4 into sulfatide of myelin, microsomal, mitochondrial and synaptosomal fractions was significantly inhibited in the jimpy mutant. In addition, the incorporation of 35SO1 into non-lipid brain membrane components (sulfated glycoproteins and mucopolysaccarides) was also decreased in jimpy mice. These results suggest that the jimpy mutation produces widespread alterations in brain sulfatide metabolism and also affects the metabolism of non-lipid membrane components of brain.