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Journal of Lipid Research, Vol. 11, 473-479, September 1970
Copyright © 1970 by Lipid Research, Inc.

Globoid cell leukodystrophy (Krabbe's disease): isolation of myelin with normal glycolipid composition

Yoshikatsu Eto , Kinuko Suzuki , and Kunihiko Suzuki

Departments of Neurology and Pathology (Neuropathology), University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104

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 C24:0 to C24:1 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.

Supplementary key words cerebroside • sulfatide • cerebroside-sulfatide sulfotransferase • galactocerebroside ßbeta;-galactosidase • inherited metabolic disease

Submitted on April 3, 1970
Accepted on June 11, 1970


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