Sulfatides in prenatal metachromatic leukodystrophy.
In one 21-week-old fetus with prenatally diagnosed metachromatic leukodystrophy, galactolipid contents were determined in the forebrain cortex, cerebellum, brainstem, spinal cord, and kidney and compared to an appropriate control. Spinal cord and kidney showed the highest sulfatide accumulation as a consequence of deficient cerebroside sulfatase activity. No increase, but a measurable amount of sulfatide, was detected in the forebrain. The prenatal neural sulfatides contained a high proportion of the hydroxy fatty acid component. The galactosyl ceramides were not reduced in the early stage of the demyelinating disease.
D002490:Central Nervous System / Q000032:analysis; D002855:Chromatography, Thin Layer; D005260:Female; D038983:Galactolipids; D006017:Glycolipids / Q000032:analysis; D006801:Humans; D007966:Leukodystrophy, Metachromatic / Q000151:congenital* / Q000378:metabolism; D011247:Pregnancy; D011262:Pregnancy Trimester, Second; D013433:Sulfoglycosphingolipids / Q000032:analysis*
D038983:Galactolipids; D006017:Glycolipids; D013433:Sulfoglycosphingolipids