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Therapeutic Effects of Nizubaglustat in a Mouse Model of GM2 Gangliosidosis. [PDF]
J Inherit Metab DisLandskroner K +3 more
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Inhibitors of Glucosylceramide Synthase
2023Glucosylceramide synthase can be targeted by high affinity small molecular weight inhibitors for the study of glycosphingolipid metabolism and function or for the treatment of glycosphingolipid storage disorders, including Gaucher and Fabry disease. This work is exemplified by the discovery and development of eliglustat tartrate, the first stand-alone ...
James A, Shayman +2 more
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GBA mutations, glucosylceramide and Parkinson's disease
Current Opinion in Neurobiology, 2022Mutations in GBA, which encodes the lysosomal enzyme glucocerebrosidase, are the highest genetic risk factor for Parkinson's disease (PD), although the mechanistic link between GBA mutations and PD is unknown. An attractive hypothesis is that the lipid substrate of glucocerebrosidase, glucosylceramide, accumulates in patients with PD with a GBA ...
Ivan Milenkovic +2 more
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Glucosylceramide synthase and apoptosis
Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids, 2002Glucosylceramide synthase (GCS) is an enzyme inherent to ceramide metabolism. The enzyme catalyzes the transfer of glucose to ceramide, the first committed step in glycolipid biosynthesis. Known for many years as a branch point enzyme directing synthesis of cerebrosides and gangliosides, GCS has recently been implicated in the cytotoxic response of ...
Richard J, Bleicher, Myles C, Cabot
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Immunogenic properties of glucosylceramide
Molecular Immunology, 1979Abstract Specific antibodies against glucosylceramide were elicited in rabbits by repeated intravenous injection of a mixture of hapten-cholesterol-human albumin (0.2:1:4) (mg/ml). The antisera obtained were titrated both by complement fixation and by passive agglutination.
B, Zalc +3 more
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Glucosylceramide synthase and glycosphingolipid synthesis
Trends in Cell Biology, 1998In mammalian cells, there are two major classes of sphingolipids---sphingomyelin and glycosphingolipids (GSLs)--both of which are synthesized from the hydrophobic molecule ceramide. The synthesis of most GSLs begins with glucosylation of ceramide to form glucosylceramide (GlcCer), which, in turn, serves as the source of 300-400 GSLs.
S, Ichikawa, Y, Hirabayashi
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2010
Glucosylceramide has a unique and often ambiguous role in mammalian cells. Activation of glucosylceramide synthase, the enzyme that places a glucosyl moiety onto ceramide, is the first pathway-committed step to the production of more complex glycosphingolipids such as lactosylceramide and gangliosides.
Maria C, Messner, Myles C, Cabot
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Glucosylceramide has a unique and often ambiguous role in mammalian cells. Activation of glucosylceramide synthase, the enzyme that places a glucosyl moiety onto ceramide, is the first pathway-committed step to the production of more complex glycosphingolipids such as lactosylceramide and gangliosides.
Maria C, Messner, Myles C, Cabot
openaire +2 more sources