Sulfatide synthesis in mice with defective synthesis of 3'-phosphoadenosine 5'-phosphosulfate

The regulation of sulfation of galactocerebroside was examined in several tissues from brachymorphic mice, including the central nervous system and kidney. These animals have an inherited defect in the enzymes which synthesize 3'-phosphoadenosine 5'-phosphosulfate (PAPS) resulting in decreased sulfation of glycosaminoglycans. In contrast, sulfation of galactocerebroside to form sulfatide (sulfogalactocerebroside) was normal in brachymorphic mice even in tissues with decreased ability to synthesize PAPS. This suggests that, unlike the regulation of glycosaminoglycan sulfation, the formation of sulfatide is not stringently regulated by tissue levels of PAPS.