Results 51 to 60 of about 5,162 (179)

Anti-Gb3 monoclonal antibody inhibits angiogenesis and tumor development.

PLoS ONE, 2012
Inhibiting the growth of tumor vasculature represents one of the relevant strategies against tumor progression. Between all the different pro-angiogenic molecular targets, plasma membrane glycosphingolipids have been under-investigated.
Ariane Desselle, Tanguy Chaumette, Marie-Hélène Gaugler, Denis Cochonneau, Julien Fleurence, Nolwenn Dubois, Philippe Hulin, Jacques Aubry, Stéphane Birklé, François Paris   +9 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

Distributions of Globotriaosylceramide Isoforms, and Globotriaosylsphingosine and Its Analogues in an α-Galactosidase A Knockout Mouse, a Model of Fabry Disease.

PLoS ONE, 2015
Fabry disease is caused by deficient activity of α-galactosidase A (GLA) and characterized by systemic accumulation of glycosphingolipids, substrates of the enzyme.
Hideaki Sueoka, Mikio Aoki, Takahiro Tsukimura, Tadayasu Togawa, Hitoshi Sakuraba   +4 more
doaj   +1 more source

Generation of a gene-corrected human isogenic iPSC line from a patient with Fabry disease carrying the GLA variant c.1069C>T using CRISPR/Cas9-mediated homology directed repair

Stem Cell Research
Fabry disease (FD) is an X-linked genetic disorder caused by mutations in the GLA gene, leading to α-galactosidase A deficiency and intracellular globotriaosylceramide (Gb3) accumulation.
Franziska Karl-Schöller, Maximilian Breyer, Eva Klopocki, Nurcan Üçeyler   +3 more
doaj   +1 more source

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
Fabry disease is an X-linked metabolic disorder due to a pathogenic mutation of the GLA gene. The accumulation of globotriaosylceramide (Gb3) damages multiple organs, including the heart, kidney and nervous system, especially in classical type Fabry ...
Kenichi Hongo, Toru Harada, Eiko Fukuro, Masahisa Kobayashi, Toya Ohashi, Yoshikatsu Eto   +5 more
doaj   +1 more source

Gb3-binding lectins as potential carriers for transcellular drug delivery

, 2016
Objectives: Epithelial cell layers as well as endothelia forming the blood-brain barrier can drastically reduce the efficiency of drug targeting. Our goal was to investigate lectins recognizing the glycosphingolipid globotriaosylceramide (Gb3) for their ...
Josef Madl (3588917), Eierhoff, Thorsten, Römer, Winfried, Katharina Zittlau (3588914), Schubert, Thomas, Winfried Römer (261968), Roland Thuenauer (509295), Zittlau, Katharina, Madl, Josef, Thuenauer, Roland, Thorsten Eierhoff (242299), Isabel Wilhelm (3588908), Thomas Schubert (394821), Imberty, Anne, Müller, Stefan K., Wilhelm, Isabel, Thünauer, Roland, Anne Imberty (75942), Stefan K. Müller (3588911)   +18 more
core   +1 more source

Determination of globotriaosylceramide in plasma and urine by mass spectrometry [PDF]

, 2009
Background: Fabry disease is an X-chromosomally inherited lysosomal storage disorder leading to accumulation of glycosphingolipids, mainly globotriaosylceramide (ceramide-trihexoside, Gb3).
Grünhage, Silke, Beck, Michael, Lackner, Karl J., Rossmann, Heidi, Bruns, Kai, Reinke, Jörg, Krüger, Ralf   +6 more
core   +1 more source

The glycosphingolipid globotriaosylceramide in the metastatic transformation of colon cancer [PDF]

, 2005
The most devastating aspect of cancer is the emergence of metastases. Thus, identification of potentially metastatic cells among a tumor cell population and the underlying molecular changes that switch cells to a metastatic state are among the most ...
Elowsky, Christian, B. Baibakov, Wang, Thomas, Kane, Anne, O. Kovbasnjuk, Kovbasnjuk, Olga, Mourtazina, Rakhilya, C. Elowsky, Donowitz, Mark, M. Donowitz, M. A. Choti, A. Kane, Choti, Michael A., Baibakov, Boris, R. Mourtazina, T. Wang   +15 more
core   +1 more source

The importance of a multidisciplinary approach in two tricky cases: the perfect match for Fabry disease

BMC Nephrology
Anderson-Fabry disease (AFD) is a multisystem X-linked lysosomal storage disorder caused by a deficiency in the enzyme α-galactosidase A (α-Gal A). This deficiency results in the intracellular accumulation of glycosphingolipids, primarily uncleaved ...
Gian Marco Berti, Valeria Aiello, Gisella Vischini, Sarah Lerario, Francesca Ciurli, Marisa Santostefano, Vincenzo Donadio, Elena Biagini, Michela Fresina, Benedetta Fabbrizio, Francesca Montanari, Daniela Turchetti, Gianandrea Pasquinelli, Renzo Mignani, Gaetano La Manna, Irene Capelli   +15 more
doaj   +1 more source

AAV2/6 Gene Therapy in a Murine Model of Fabry Disease Results in Supraphysiological Enzyme Activity and Effective Substrate Reduction

Molecular Therapy: Methods & Clinical Development, 2020
Fabry disease is an X-linked lysosomal storage disorder caused by mutations in the alpha-galactosidase A (GLA) gene, which encodes the exogalactosyl hydrolase, alpha-galactosidase A (α-Gal A).
Makiko Yasuda, Marshall W. Huston, Silvere Pagant, Lin Gan, Susan St. Martin, Scott Sproul, Daniel Richards, Stephen Ballaron, Khaled Hettini, Annemarie Ledeboer, Lillian Falese, Liching Cao, Yanmei Lu, Michael C. Holmes, Kathleen Meyer, Robert J. Desnick, Thomas Wechsler   +16 more
doaj   +1 more source