Journal of Lipid Research, Vol. 15, 227-233, May 1974
Copyright © 1974 by Lipid Research, Inc.

Structure and composition of sulfatides isolated from livers of patients with metachromatic leukodystrophy: galactosyl sulfatide and lactosyl sulfatide

Mutsumi Sugita , John T. Dulaney , and Hugo W. Moser

Eunice Kennedy Shriver Center, Walter E. Fernald State School, Waltham, Massachusetts 02154

The livers of four patients with metachromatic leukodystrophy contained galactosyl sulfatide and lactosyl sulfatide, whereas these substances were undetectable in normal human liver. On the basis of methanolysis and permethylation studies, both sulfatides were shown to be substituted with sulfate at the C-3 position of the galactose moiety. Examination of the fatty acid compositions of these sulfatides showed that C_22:0 and higher 2-hydroxy and nonhydroxy fatty acids predominated in both. Both sulfatides contained the same long-chain bases, predominantly sphingosine, dihydrosphingosine, and phytosphingosine. Using as criteria the proportion of lactosyl sulfatide to galactosyl sulfatide, and the fatty acid and long-chain base compositions, the liver sulfatides from subjects with metachromatic leukodystrophy closely resemble those in the kidney and differ from those in brain and peripheral nerve.

Supplementary key words fatty acids • sphingosines • hexose • gas-liquid chromatography

Submitted on August 22, 1973
Accepted on January 15, 1974

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