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Structure and composition of sulfatides isolated from livers of patients with metachromatic leukodystrophy: galactosyl sulfatide and lactosyl sulfatide
- 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.
- Subjects :
- chemistry.chemical_classification
Kidney
Sphingosine
Leukodystrophy
gas–liquid chromatography
Fatty acid
QD415-436
Cell Biology
medicine.disease
fatty acids
Biochemistry
Metachromatic leukodystrophy
chemistry.chemical_compound
Endocrinology
medicine.anatomical_structure
chemistry
Galactose
medicine
hexose
Hexose
Composition (visual arts)
sphingosines
Subjects
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