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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Main Authors: Mutsumi Sugita, John T. Dulaney, Hugo W. Moser
Format: Article
Language:English
Published: Elsevier 1974-05-01
Series:Journal of Lipid Research
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0022227520368000
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spelling 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
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