Results 11 to 20 of about 9,362 (180)
Cells, 2021 Substrate reduction therapy (SRT) in clinic adequately manages the visceral manifestations in Gaucher disease (GD) but has no direct effect on brain disease.
Yanyan Peng +8 more
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Human Brain Lipidomics: Investigation of Formalin Fixed Brains
Frontiers in Molecular Neuroscience, 2022 Human brain lipidomics have elucidated structural lipids and lipid signal transduction pathways in neurologic diseases. Such studies have traditionally sourced tissue exclusively from brain bank biorepositories, however, limited inventories signal that ...
Aaron W. Beger +5 more
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Property-based design of a glucosylceramide synthase inhibitor that reduces glucosylceramide in the brain [PDF]
Journal of Lipid Research, 2012 Synthesis inhibition is the basis for the treatment of type 1 Gaucher disease by the glucosylceramide synthase (GCS) inhibitor eliglustat tartrate. However, the extended use of eliglustat and related compounds for the treatment of glycosphingolipid storage diseases with CNS manifestations is limited by the lack of brain penetration of this drug ...
Scott D. Larsen +9 more
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The fate and function of glycosphingolipid glucosylceramide [PDF]
Philosophical Transactions of the Royal Society of London. Series B: Biological Sciences, 2003 In higher eukaryotes, glucosylceramide is the simplest member and precursor of a fascinating class of membrane lipids, the glycosphingolipids. These lipids display an astounding variation in their carbohydrate head groups, suggesting that glycosphingolipids serve specialized functions in recognition processes. It is now realized that they are organized
van Meer, G. +2 more
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Journal of Lipid Research, 2000 In the present study, 2,2,6,6-tetramethylpiperidinooxy nitroxide (TEMPO) has been applied successfully to discriminate between glucosylceramide in the outer and inner leaflets of closed membrane bilayers.
Daniel J. Sillence +4 more
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The role of glucosylceramide and glucosylceramide synthase in liver disease: from bench to bedside
Acta Biochimica Polonica, 2020 The cell membrane, which is lipid-rich, is not only a simple mechanical barrier but also an important and complex component of the cell. It also communicates with the external environment. Sphingomyelin is an important class of phospholipids in the membrane that performs many functions.
Jian Gan +3 more
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Lysosomal membrane integrity in fibroblasts derived from patients with Gaucher disease
Cell Structure and Function, 2023 Gaucher disease (GD) is a recessively inherited lysosomal storage disorder characterized by a deficiency of lysosomal glucocerebrosidase (GBA1). This deficiency results in the accumulation of its substrate, glucosylceramide (GlcCer), within lysosomes ...
Asuka Hamamoto +7 more
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Improved Inhibitors of Glucosylceramide Synthase [PDF]
Journal of Biological Chemistry, 1999 Previous work has led to the identification of inhibitors of glucosylceramide synthase, the enzyme catalyzing the first glycosylation step in the synthesis of glucosylceramide-based glycosphingolipids. These inhibitors have two identified sites of action: the inhibition of glucosylceramide synthase, resulting in the depletion of cellular ...
L, Lee, A, Abe, J A, Shayman
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Molecules, 2020 Cerebrosides (Crb; including glucosylceramide and galactosylceramide) and lactosylceramide (LacCer) are structurally complex lipids found in many eukaryotic cell membranes, where they play important roles in cell growth, apoptosis, cell recognition and ...
Lin Ma +3 more
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Journal of Lipid Research, 1987 The time course of the clearance from the blood and the tissue localization of [14C]L-glucosylceramide, a nonmetabolizable enantiomorph of D-glucosylceramide that accumulates in Gaucher's disease, has been determined.
T Tokoro, A E Gal, L L Gallo, R O Brady
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