Results 91 to 100 of about 633 (112)
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Exogenous Galactosylceramide as Potential Treatment for CLN3 Disease

Annals of Neurology, 2019
ObjectiveCLN3 disease is the commonest of the neuronal ceroid lipofuscinoses, a group of pediatric neurodegenerative disorders. Functions of the CLN3 protein include antiapoptotic properties and facilitating anterograde transport of galactosylceramide from Golgi to lipid rafts.
Sally El‐Sitt   +9 more
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Immunostimulatory activities of mono- or diglycosylated α-galactosylceramides

Bioorganic & Medicinal Chemistry, 1997
We examined the effects of 2"- or 3"-monoglycosylated alpha-galactosylceramides (alpha-GalCers) and 2",3"-diglycosylated alpha-GalCers on allogeneic MLR and the proliferation of murine spleen cells. It was found that their ceramide portions greatly affect their immunostimulatory activities, and that the 3"-hydroxyl group plays a more important role in ...
A, Uchimura   +7 more
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Synthesis of galactosylphytosphingosine and galactosylceramides containing phytosphingosine

Chemistry and Physics of Lipids, 1974
Abstract Phytosphingosine prepared from the yeast Hansenula ciferrii was used for synthesis of galactosphingolipids. Glycosidation of N -dichloroacetyl and 3,4-di- O -benzoyl protected phytosphingosine was performed with acetobromogalactose in the presence of mercury cyanide.
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Structural determinants of miscibility in surface films of galactosylceramide and phosphatidylcholine: effect of unsaturation in the galactosylceramide acyl chain

Biochemistry, 1991
The Langmuir film balance technique has been used to define the surface structure and determine the mixing behavior of galactosylceramide (GalCer) and phosphatidylcholines in surface phases. To determine the effect of unsaturation on surface behavior, chain-pure GalCer species containing either oleoyl (18:1 delta 9), eicosenoyl (20:1 delta 11), or ...
S, Ali, H L, Brockman, R E, Brown
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α‐Galactosylceramide activates antitumor immunity against liver tumor

Hepatology Research, 2010
Aim:  α‐Galactosylceramide (α‐GalCer) has been attracting attention as a novel approach to treat metastatic liver cancer. We investigated the detailed process of activating liver dendritic cells (DC) and immune cells after α‐GalCer treatment in the mouse liver tumor model.Methods:  BALB/c mice bearing CMS4 liver tumor (p53 peptide‐expressing tumor ...
Tomohide, Tatsumi   +8 more
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Use of Mixed Dispersion of Fluorescent Galactosylceramide and Sodium Dodecylsulfate for Assaying Galactosylceramide-β-Galactosidase and Diagnosing Krabbe Disease

Enzyme, 1985
A fluorescent derivative of galactosylceramide [NBD-GalCer: 12-N-methyl-N-(7-nitrobenz-2-oxa-1,3-diazol-4-yl)aminodecanoyl+ ++ galactosylceramide] was dispersed in sodium dodecylsulfate and used to determine the activity of galactosylceramide-beta-galactosidase.
R, Salvayre, S, Gatt
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Drastically Abnormal Gluco- and Galactosylceramide Composition Does Not Affect Ganglioside Metabolism in the Brain of Mice Deficient in Galactosylceramide Synthase

Neurochemical Research, 1999
Mice that are genetically deficient in UDP-galactose: ceramide galactosyltransferase are unable to synthesize galactosylceramide. Consequently, sulfatide, which can be synthesized only by sulfation of galactosylceramide, is also totally absent in affected mouse brain.
K, Suzuki   +3 more
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Glucosylceramide and Galactosylceramide Synthase

2006
Most mammalian glycosphingolipid formation begins with the synthesis of cerebrosides, glucosylceramide and galactosylceramide. The synthases catalyzing the formations of glucosylceramide (GlcCer) and galactosylceramide (GalCer) have been characterized at the gene and protein levels.
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Metabolism of exogenous galactosylceramide in the twitcher mouse brain

Neurochemical Research, 1989
The in vivo metabolism of galactosylceramide (gal-cer) in normal mice and in twitcher mice, a model of human GLD, was examined following intracerebral administration of gal-cer containing [1-14C] stearic acid. In normal mice, gal-cer was hydrolyzed to ceramide within 6 hours and ceramide was hydrolyzed to sphingosine and fatty acid.
K, Mitsuo   +3 more
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Hepatic galactosylceramide in globoid cell leukodystrophy (Krabbe's disease)

Lipids, 1973
AbstractGlycosphingolipids were isolated from the liver of four patients with globoid cell leukodystrophy and compared to those from normal liver. The ratio of glucosylceramide to galactosylceramide was 0.8, 1.2, 1.3 and 7.5 in the four cases studied, compared to a value of greater than 10 in liver from control patients of the same age and sex.
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