Structure and composition of sulfatides isolated from livers of patients with metachromatic leukodystrophy: galactosyl sulfatide and lactosyl sulfatide
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...
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1974-05-01
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doaj-6e67a79e5dd24c6da8f867397498fd142021-04-24T05:49:12ZengElsevierJournal of Lipid Research0022-22751974-05-01153227233Structure and composition of sulfatides isolated from livers of patients with metachromatic leukodystrophy: galactosyl sulfatide and lactosyl sulfatideMutsumi Sugita0John T. Dulaney1Hugo W. Moser2Eunice Kennedy Shriver Center, Walter E. Fernald State School, Waltham, Massachusetts 02154Eunice Kennedy Shriver Center, Walter E. Fernald State School, Waltham, Massachusetts 02154Eunice Kennedy Shriver Center, Walter E. Fernald State School, Waltham, Massachusetts 02154The 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 C22:0 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.http://www.sciencedirect.com/science/article/pii/S0022227520368000fatty acidssphingosineshexosegas–liquid chromatography |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Mutsumi Sugita John T. Dulaney Hugo W. Moser |
spellingShingle |
Mutsumi Sugita John T. Dulaney Hugo W. Moser Structure and composition of sulfatides isolated from livers of patients with metachromatic leukodystrophy: galactosyl sulfatide and lactosyl sulfatide Journal of Lipid Research fatty acids sphingosines hexose gas–liquid chromatography |
author_facet |
Mutsumi Sugita John T. Dulaney Hugo W. Moser |
author_sort |
Mutsumi Sugita |
title |
Structure and composition of sulfatides isolated from livers of patients with metachromatic leukodystrophy: galactosyl sulfatide and lactosyl sulfatide |
title_short |
Structure and composition of sulfatides isolated from livers of patients with metachromatic leukodystrophy: galactosyl sulfatide and lactosyl sulfatide |
title_full |
Structure and composition of sulfatides isolated from livers of patients with metachromatic leukodystrophy: galactosyl sulfatide and lactosyl sulfatide |
title_fullStr |
Structure and composition of sulfatides isolated from livers of patients with metachromatic leukodystrophy: galactosyl sulfatide and lactosyl sulfatide |
title_full_unstemmed |
Structure and composition of sulfatides isolated from livers of patients with metachromatic leukodystrophy: galactosyl sulfatide and lactosyl sulfatide |
title_sort |
structure and composition of sulfatides isolated from livers of patients with metachromatic leukodystrophy: galactosyl sulfatide and lactosyl sulfatide |
publisher |
Elsevier |
series |
Journal of Lipid Research |
issn |
0022-2275 |
publishDate |
1974-05-01 |
description |
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 C22:0 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. |
topic |
fatty acids sphingosines hexose gas–liquid chromatography |
url |
http://www.sciencedirect.com/science/article/pii/S0022227520368000 |
work_keys_str_mv |
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