Globoid cell leukodystrophy (Krabbe's disease): isolation of myelin with normal glycolipid composition.
Myelin was isolated from the brain of a patient with Krabbe's globoid cell leukodystrophy at 0.4% of the normal yield. Despite the exceedingly low yield, the fraction appeared morphologically clean, and consisted mostly of well-preserved myelin lamellae and few contaminating structures. Total lipid and cholesterol were slightly lower than in normal myelin. Total phospholipid was normal, but the ratio of ethanolamine phospholipid to lecithin was reversed. Total galactolipid was normal, and consisted only of cerebroside and sulfatide in normal proportions. The only sugar in cerebroside and sulfatide was galactose. The fatty acid composition of cerebroside and sulfatide was essentially normal with no deficiency of long-chain fatty acids and only with a reversed ratio of C(24:0) to C(24:1) in cerebroside. These data appear to exclude the previous postulate that abnormally rapid breakdown of myelin occurs in this disorder as the result of the formation of chemically abnormal myelin, deficient in sulfatide.
D001923:Brain Chemistry; D002499:Centrifugation, Density Gradient; D002554:Cerebrosides / Q000032:analysis; D002784:Cholesterol / Q000032:analysis; D002855:Chromatography, Thin Layer; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000032:analysis / Q000235:genetics*; D005227:Fatty Acids / Q000032:analysis; D005690:Galactose / Q000032:analysis; D006017:Glycolipids / Q000032:analysis*; D006801:Humans; D007223:Infant; D008055:Lipids / Q000032:analysis; D008297:Male; D008854:Microscopy, Electron; D009186:Myelin Sheath / Q000032:analysis*; D010743:Phospholipids / Q000032:analysis; D013464:Sulfuric Acids / Q000032:analysis
D002554:Cerebrosides; D005227:Fatty Acids; D006017:Glycolipids; D008055:Lipids; D010743:Phospholipids; D013464:Sulfuric Acids; D002784:Cholesterol; D005690:Galactose