Results 41 to 50 of about 7,739 (261)

Massive accumulation of globotriaosylceramide in various tissues from a Fabry patient with a high antibody titer against alpha-galactosidase A after 6 years of enzyme replacement therapy

Molecular Genetics and Metabolism Reports, 2020
Kenichi Hongo, Toru Harada, Eiko Fukuro, Masahisa Kobayashi, Toya Ohashi, Yoshikatsu Eto   +5 more
openalex   +2 more sources

Dysregulation of Immune Response Mediators and Pain-Related Ion Channels Is Associated with Pain-like Behavior in the GLA KO Mouse Model of Fabry Disease

Cells, 2022
Fabry disease (FD) is a rare life-threatening disorder caused by deficiency of the alpha-galactosidase A (GLA) enzyme with a characteristic pain phenotype.
Marlene Spitzel, Elise Wagner, Maximilian Breyer, Dorothea Henniger, Mehtap Bayin, Lukas Hofmann, Daniela Mauceri, Claudia Sommer, Nurcan Üçeyler   +8 more
doaj   +1 more source

A Shiga Toxin B-Subunit-Based Lectibody Boosts T Cell Cytotoxicity towards Gb3-Positive Cancer Cells

Cells, 2023
Aberrant glycosylation plays a crucial role in tumour progression and invasiveness. Tumour-associated carbohydrate antigens (TACAs) represent a valuable set of targets for immunotherapeutic approaches.
Jana Tomisch, Vincent Busse, Francesca Rosato, Olga N. Makshakova, Pavel Salavei, Anna-Sophia Kittel, Emilie Gillon, Levin Lataster, Anne Imberty, Ana Valeria Meléndez, Winfried Römer   +10 more
doaj   +1 more source

Inhibition of tumor angiogenesis by globotriaosylceramide immunotargeting [PDF]

OncoImmunology, 2013
Current antiangiogenic immunotherapeutic strategies mainly focus on the blockade of circulating cytokines or receptors that are overexpressed by endothelial cells. We proposed globotriaosylceramide (Gb3) as a viable alternative target for antiangiogenic therapies. In this setting, we developed an anti-Gb3 antibody and validated its therapeutic efficacy
Birklé, S., Desselle, A., Chaumette, T., Gaugler, M.-H., Cochonneau, D., Fleurence, J., Dubois, N., Hulin, P., Aubry, J., Paris, F.   +9 more
openaire   +3 more sources

Shiga Toxin-Bearing Microvesicles Exert a Cytotoxic Effect on Recipient Cells Only When the Cells Express the Toxin Receptor

Frontiers in Cellular and Infection Microbiology, 2020
Shiga toxin is the main virulence factor of non-invasive enterohemorrhagic Escherichia coli strains capable of causing hemolytic uremic syndrome. Our group has previously shown that the toxin can reach the kidney within microvesicles where it is taken up
Karl Johansson, Annie Willysson, Ann-Charlotte Kristoffersson, Ashmita Tontanahal, Daniel Gillet, Anne-lie Ståhl, Diana Karpman   +6 more
doaj   +1 more source

Shiga Toxin Uptake and Sequestration in Extracellular Vesicles Is Mediated by Its B-Subunit

Toxins, 2020
Shiga toxin (Stx)-stimulated blood cells shed extracellular vesicles (EVs) which can transfer the toxin to the kidneys and lead to hemolytic uremic syndrome.
Annie Willysson, Anne-lie Ståhl, Daniel Gillet, Julien Barbier, Jean-Christophe Cintrat, Valérie Chambon, Anne Billet, Ludger Johannes, Diana Karpman   +8 more
doaj   +1 more source

Structural Diversities of Lectins Binding to the Glycosphingolipid Gb3

Frontiers in Molecular Biosciences, 2021
Glycolipids are present on the surfaces of all living cells and thereby represent targets for many protein receptors, such as lectins. Understanding the interactions between lectins and glycolipids is essential for investigating the functions of lectins ...
Lina Siukstaite, Lina Siukstaite, Anne Imberty, Winfried Römer, Winfried Römer, Winfried Römer   +5 more
doaj   +1 more source

Fabry disease – a multisystemic disease with gastrointestinal manifestations

Gut Microbes, 2022
Nonspecific gastrointestinal (GI) symptoms, such as postprandial cramping pain, diarrhea, nausea and vomiting are typical symptoms for irritable bowel syndrome or inflammatory bowel disease, but may also be the first symptoms of Fabry disease (FD).
Malte Lenders, Eva Brand
doaj   +1 more source

Enzymatic Glyco-Modification of Synthetic Membrane Systems

Biomolecules, 2023
The present report assesses the capability of a soluble glycosyltransferase to modify glycolipids organized in two synthetic membrane systems that are attractive models to mimic cell membranes: giant unilamellar vesicles (GUVs) and supported lipid ...
Dylan Jabeguero, Lina Siukstaite, Chunyue Wang, Anna Mitrovic, Serge Pérez, Olga Makshakova, Ralf P. Richter, Winfried Römer, Christelle Breton   +8 more
doaj   +1 more source

Inhibition of Multidrug Resistance by AdamantylGb3, a Globotriaosylceramide Analog [PDF]

Journal of Biological Chemistry, 2008
Multidrug resistance (MDR) via the ABC drug transporter (ABCB1), P-glycoprotein (P-gp/MDR1) overexpression, is a major obstacle in cancer chemotherapy. Many inhibitors reverse MDR but, like cyclosporin A (CsA), have significant toxicities. MDR1 is also a translocase that flips glucosylceramide inside the Golgi to enhance neutral glycosphingolipid (GSL)
María Fabiana, De Rosa, Cameron, Ackerley, Bernice, Wang, Shinya, Ito, David M, Clarke, Clifford, Lingwood   +5 more
openaire   +2 more sources