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

Authors :
John T. Dulaney
Mutsumi Sugita
Hugo W. Moser
Source :
Journal of Lipid Research, Vol 15, Iss 3, Pp 227-233 (1974)
Publication Year :
1974
Publisher :
Elsevier BV, 1974.

Abstract

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.

Details

ISSN :
00222275
Volume :
15
Database :
OpenAIRE
Journal :
Journal of Lipid Research
Accession number :
edsair.doi.dedup.....0b244d89cd1c392057efbcc50033cd36
Full Text :
https://doi.org/10.1016/s0022-2275(20)36800-0