Myelin Membrane: a molecular abnormality.

Myelin was isolated from cerebral white matter from a patient who had died of metachromatic leukodystrophy, and its lipid composition was analyzed. Although the lipid content was nearly normal, the myelin contained a three- to fourfold excess of cerebroside sulfate and a threefold deficiency of cerebroside compared to normal myelin. The deficiency of cerebroside and the excess of cerebroside sulfate may account for defective myelination in this disease.