Results 21 to 30 of about 2,629 (119)

Studies of the pathogenesis of Gaucher's disease: tissue distribution and biliary excretion of [14C]L-glucosylceramide in rats

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
doaj   +1 more source

β-Glucocerebrosidase Deficiency Activates an Aberrant Lysosome-Plasma Membrane Axis Responsible for the Onset of Neurodegeneration

Cells, 2022
β-glucocerebrosidase is a lysosomal hydrolase involved in the catabolism of the sphingolipid glucosylceramide. Biallelic loss of function mutations in this enzyme are responsible for the onset of Gaucher disease, while monoallelic β-glucocerebrosidase ...
Giulia Lunghi, Emma Veronica Carsana, Nicoletta Loberto, Laura Cioccarelli, Simona Prioni, Laura Mauri, Rosaria Bassi, Stefano Duga, Letizia Straniero, Rosanna Asselta, Giulia Soldà, Alessio Di Fonzo, Emanuele Frattini, Manuela Magni, Nara Liessi, Andrea Armirotti, Elena Ferrari, Maura Samarani, Massimo Aureli   +18 more
doaj   +1 more source

Intestinal absorption of dietary maize glucosylceramide in lymphatic duct cannulated rats

Journal of Lipid Research, 2010
Sphingolipids are ubiquitous in all eukaryotic organisms. Various physiological functions of dietary sphingolipids, such as preventing colon cancer and improving the skin barrier function, have been recently reported. One of the common sphingolipids used
Tatsuya Sugawara, Tsuyoshi Tsuduki, Saeko Yano, Mayumi Hirose, Jingjing Duan, Kazuhiko Aida, Ikuo Ikeda, Takashi Hirata   +7 more
doaj   +1 more source

Role of lactosylceramide and MAP kinase in the proliferation of proximal tubular cells in human polycystic kidney disease.

Journal of Lipid Research, 1996
Polycystic kidney disease (PKD) is a common genetic disease characterized by the proliferation of epithelial cells, formation of cysts, and the progression of renal deficiency.
S Chatterjee, W Y Shi, P Wilson, A Mazumdar   +3 more
doaj   +1 more source

Glucosylceramide is synthesized at the cytosolic surface of various Golgi subfractions [PDF]

, 1992
In our attempt to assess the topology of glucosylceramide biosynthesis, we have employed a truncated ceramide analogue that permeates cell membranes and is converted into water soluble sphingolipid analogues both in living and in fractionated cells ...
Jeckel, D., Karrenbauer, A., Wieland, F., G van Meer, Burger, K.N.J., van Meer, G., KN Burger, F Wieland, D Jeckel, A Karrenbauer   +9 more
core   +3 more sources

Glucosylceramide Plays a Role in Fungal Germination, Lipid Raft Organization and Biofilm Adhesion of the Pathogenic Fungus Scedosporium aurantiacum

Journal of Fungi, 2020
Infections caused by Scedosporium species present a wide range of clinical manifestations, from superficial to disseminated, especially in immunocompromised patients.
Victor Pereira Rochetti, Rodrigo Rollin-Pinheiro, Evely Bertulino de Oliveira, Mariana Ingrid Dutra da Silva Xisto, Eliana Barreto-Bergter   +4 more
doaj   +1 more source

Structural diversity and biological significance of glycosphingolipids in pathogenic and opportunistic fungi

Frontiers in Cellular and Infection Microbiology, 2014
Glycosphingolipids (GSLs) are ubiquitous membrane components and have key roles in biological systems, acting as second messengers or modulators of signal transduction by affecting several events, ranging from cell adhesion, cell growth, cell motility ...
Luciana Lopes Guimaraes, Luciana Lopes Guimaraes, Marcos Sergio Toledo, Felipe Araujo Souza Ferreira, Anita Hilda Straus, Helio K Takahashi   +5 more
doaj   +1 more source

Unraveling A4GALT Mechanism and Its Modulation With Adamantyl‐Galactosylceramide Analogues: Advancing Fabry Disease Therapeutic Strategies

Angewandte Chemie, Volume 138, Issue 41, 5 October 2026.
A 310‐helix‐mediated conformational switch promotes a front‐face SNi‐like catalysis by human A4GALT. Mechanism‐guided design identifies AdaGalCer as a selective modulator of globotriaosylceramide (Gb3) biosynthesis, opening a clear route toward new Fabry disease therapeutics.
Nicky de Koster, Òscar Vidal‐Gironès, Rowan de Graaf, Ken Kok, Lindsey T. Lelieveld, Jackelien Hoogervorst, Maria J. Ferraz, José Pablo Rivas‐Fernández, Rob F. Lammers, Herman S. Overkleeft, Johannes M. F. G. Aerts, Rolf G. Boot, Carme Rovira, Marta Artola   +13 more
wiley   +2 more sources

Re-Configuration of Sphingolipid Metabolism by Oncogenic Transformation

Biomolecules, 2014
The sphingolipids are one of the major lipid families in eukaryotes, incorporating a diverse array of structural variants that exert a powerful influence over cell fate and physiology. Increased expression of sphingosine kinase 1 (SPHK1), which catalyses
Anthony S. Don, Xin Y. Lim, Timothy A. Couttas   +2 more
doaj   +1 more source

The development and use of small molecule inhibitors of glycosphingolipid metabolism for lysosomal storage diseases

Journal of Lipid Research, 2014
Glycosphingolipid (GSL) storage diseases have been the focus of efforts to develop small molecule therapeutics from design, experimental proof of concept studies, and clinical trials.
James A. Shayman, Scott D. Larsen
doaj   +1 more source