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 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.

[1]  P. Mandel,et al.  PAPS—cerebroside sulphotransferase activity in brain and kidney of neurological mutants , 1971, FEBS letters.

[2]  C. Meurant,et al.  Human urinary sulfatides in patients with sulfatidosis (metachromatic leukodystrophy). , 1971, Journal of lipid research.

[3]  R. Brady,et al.  Metachromatic Leukodystrophy: Diagnosis with Samples of Venous Blood , 1968, Science.

[4]  M. Kamimura,et al.  Biochemistry of shellfish lipids. 8. Occurrence of ceramide mono- and dihexoside in corbicula, Corbicula sandai. , 1968, Journal of biochemistry.

[5]  A. Stoffyn,et al.  Structure of kidney ceramide dihexoside sulfate. , 1968, Biochimica et biophysica acta.

[6]  S. Ställberg-Stenhagen,et al.  Changes in the fatty acid composition of cerebrosides and sulfatides of human nervous tissue with age. , 1968, Journal of lipid research.

[7]  K. Karlsson,et al.  Studies on sphingosines. XIV. On the phytosphingosine content of the major human kidney glycolipids. , 1968, Biochimica et biophysica acta.

[8]  H. Greene,et al.  Arylsulfatase A in the urine and metachromatic leukodystrophy. , 1967, The Journal of pediatrics.

[9]  H. Moser,et al.  The dynamics of a lipidosis. Turnover of sulfatide, steroid sulfate, and polysaccharide sulfate in metachromatic leukodystrophy. , 1967, Archives of neurology.

[10]  G. M. Gray,et al.  The structure of the oligosaccharide moieties of pig lung glycolipids , 1967 .

[11]  G. Mckhann,et al.  THE IN VIVO AND IN VITRO SYNTHESIS OF SULPHATIDES DURING DEVELOPMENT * , 1967, Journal of neurochemistry.

[12]  M. Malone,et al.  STRUCTURAL STUDIES ON SULPHATIDES IN METACHROMATIC LEUCODYSTROPHY , 1966, Journal of neurochemistry.

[13]  M. Philippart,et al.  Concentration and fatty acid composition of cerebrosides and sulfatides in mature and immature human brain. , 1966, Journal of lipid research.

[14]  E. Mårtensson.  Sulfatides of human kidney. Isolation, identification, and fatty acid composition. , 1966, Biochimica et biophysica acta.

[15]  D. Armstrong,et al.  Metachromatic form of diffuse cerebral sclerosis. V. The nature and significance of low sulfatase activity: a controlled study of brain, liver and kidney in four patients with metachromatic leukodystrophy (MLD). , 1965, Archives of neurology.

[16]  J. O'brien,et al.  Fatty acid and fatty aldehyde composition of the major brain lipids in normal human gray matter, white matter, and myelin. , 1965, Journal of lipid research.

[17]  E. Mehl,et al.  EVIDENCE FOR THE GENETIC BLOCK IN METACHROMATIC LEUCODYSTROPHY (ML). , 1965, Biochemical and biophysical research communications.

[18]  M. Malone,et al.  STRUCTURAL STUDIES ON SULFATIDES IN METACHROMATIC LEUKODYSTROPHY. , 1965, Biochimica et biophysica acta.

[19]  G. Rouser,et al.  The fatty acid composition of brain sphingolipids: sphingomyelin, ceramide, cerebroside, and cerebroside sulfate. , 1964, Journal of lipid research.

[20]  J. O'brien,et al.  A molecular defect of myelination. , 1964, Biochemical and biophysical research communications.

[21]  S. Hakomori.  A RAPID PERMETHYLATION OF GLYCOLIPID, AND POLYSACCHARIDE CATALYZED BY METHYLSULFINYL CARBANION IN DIMETHYL SULFOXIDE. , 1964, Journal of biochemistry.

[22]  N. Radin,et al.  OCCURRENCE OF 2-HYDROXY FATTY ACIDS IN ANIMAL TISSUES. , 1963, Journal of lipid research.

[23]  T. Yamakawa,et al.  On the structure of brain cerebroside sulfuric ester and ceramide dihexoside of erythrocytes. , 1962, Journal of biochemistry.

[24]  M. Rapport,et al.  A simplified preparation of sphingomyelin. , 1958, The Journal of biological chemistry.

[25]  S. Aronson,et al.  Sphingolipids, Sphingolipidoses and Allied Disorders , 1972, Advances in Experimental Medicine and Biology.

[26]  W. Stoffel,et al.  Metabolism of sphinosine bases, XIX. On the origin of phytosphingosine (4d-hydroxysphinganine) in mammalian tissues. , 1972, Hoppe-Seyler's Zeitschrift fur physiologische Chemie.

[27]  H. Moser,et al.  Liver Glycolipids, Steroid Sulfates and Steroid Sulfatases in a Form of Metachromatic Leukodystrophy Associated with Multiple Sulfatase Deficiencies , 1972 .

[28]  A. Stoffyn,et al.  Direct conversion of sulfatides into cerebrosides. , 1963, Biochimica et biophysica acta.