Sulfatide

Sulfatides are hydrogen sulfate ester of glycosphingolipids. It is cerebrosides in which the C3 of the glycosyl moiety (either glucose or galactose ) a sulfate group is attached. The donor is 3'- phosphoadenosine -5'- phosphosulfate ( PAPS ). They are formed primarily in oligodendrocytes in the CNS. A defect or deficiency of sulfate -releasing Sulfatidase arylsulfatase A and the resulting accumulation of sulfatides in the lysosomes of oligodendrocytes leads to metachromatic leukodystrophy.