Results 61 to 70 of about 5,162 (179)

Glucosylceramide synthase regulates MDR1 gene expression through the association of globotriaosylceramide (Gb3) and histone acetylation

The FASEB Journal, 2009
MDR1 gene is frequently overexpressed in drug‐resistant tumor, and encodes a large amount of P‐glycoprotein protecting cells from cytotoxins. Glucosylceramide synthase (GCS) converting ceramide into glucosylceramide initiates glycosphingolipid (GSL) synthesis and defaults drug‐induced apoptosis.
Yong Yu Liu, Vineet Gupta, Gauri Anand Patwardhan, Rajeswara Pinnoji, Victor S. Hsia, S. Michal Jazwinski   +5 more
openaire   +1 more source

SGLT2 Inhibitors and Cardiovascular Outcomes in Patients With Fabry Disease: Observations From a Federated Research Network

Diabetes, Obesity and Metabolism, EarlyView.
ABSTRACT Background and Aim Fabry disease (FD) is frequently complicated by renal impairment and cardiovascular events; however, evidence on sodium–glucose cotransporter‐2 inhibitors (SGLT2i) in this population is scarce. Accordingly, we assessed the association between SGLT2i exposure and cardiovascular outcomes in adults with FD. Methods We conducted
Leandra Serio, Michele Rossi, Amir Askarinejad, Enrico Tartaglia, Tommaso Bucci, Enrica Marrone, Dinesh K. Kalra, Claudio Ferri, Gregory Y. H. Lip   +8 more
wiley   +1 more source

Inhibition of tumor angiogenesis by globotriaosylceramide immunotargeting

, 2013
International audienceCurrent antiangiogenic immunotherapeutic strategies mainly focus on the blockade of circulating cytokines or receptors that are overexpressed by endothelial cells.
Cochonneau, D., Chaumette, T., Paris, F., Birklé, S., Dubois, N., Desselle, A., Fleurence, J., Gaugler, M.-H., Hulin, P., Aubry, J.   +9 more
core   +1 more source

Sensory-specific peripheral nerve pathology in a rat model of Fabry disease

Neurobiology of Pain, 2021
Fabry disease (FD) causes life-long pain, the mechanisms of which are unclear. Patients with FD have chronic pain that mirrors symptoms of other painful peripheral neuropathies. However, it is unclear what underlying damage occurs in FD peripheral nerves
Tyler B. Waltz, Anthony J. Burand, Jr., Katelyn E. Sadler, Cheryl L. Stucky   +3 more
doaj   +1 more source

A novel soluble analog of the HIV-1 fusion cofactor, globotriaosylceramide (Gb3), eliminates the cholesterol requirement for high affinity gp120/Gb3 interaction [PDF]

Journal of Lipid Research, 2002
We have analyzed the interaction of adamantyl Gb(3) (adaGb(3)), a semi-synthetic soluble analog of Gb(3), with HIV-1 surface envelope glycoprotein gp120. In this analog, which was orginally designed to inhibit verotoxin binding to its glycolipid receptor, Gb(3), the fatty acid chain is replaced with a rigid globular hydrocarbon frame (adamantane ...
Radhia Mahfoud, Murugesapillai Mylvaganam, Clifford A. Lingwood, Jacques Fantini   +3 more
openaire   +3 more sources

Systematic Analysis of Plasma‐Derived Extracellular Vesicle‐Enriched Samples in Age‐Related Macular Degeneration Reveals Oxidative Stress Linked to Altered Lipid and Protein Profiles

Journal of Extracellular Biology, Volume 5, Issue 9, September 2026.
ABSTRACT Vision impairment caused by age‐related macular degeneration (AMD) is a global health priority. Retinal pathology is driven by ageing and exacerbated by genetic and environmental risk factors. Peripheral blood circulation is increasingly recognised as a contributor to disease initiation and progression, and a source of biomarkers to support ...
Marzena Kurzawa‐Akanbi, Jennifer Haggarty, Carina Hansohn, Mohamed T. Patel, Angela J. Cree, Peisi Teo, Milly Armstrong, Nathan Coles, Mark Platt, Helen Griffiths, Heather J. Cordell, Ahmad Khundakar, Pola Goldberg Oppenheimer, Phillip Whitfield, Andrew Lotery, Henning Urlaub, Sina Mozaffari‐Jovin, Majlinda Lako   +17 more
wiley   +1 more source

Occurrence of Infusion Associated Reactions and Antidrug Antibodies in Enzyme Replacement Therapy for Fabry Disease and the Effect of Preventive Measures

Journal of Inherited Metabolic Disease, Volume 49, Issue 5, September 2026.
Occurrence of infusion associated reactions and anti‐drug antibodies in enzyme replacement therapy for Fabry disease and the effect of preventive measures. ABSTRACT Male patients with the classical phenotype of Fabry disease (FD) are at risk of developing inhibiting antidrug antibodies (iADAs) against recombinant α‐galactosidase‐A, administered in the ...
Maud Janssens, Sanne J. van der Veen, Laura van Dussen, André B. P. van Kuilenburg, Marion M. M. G. Brands, Mirjam Langeveld   +5 more
wiley   +1 more source

Systematic gene therapy derived from an investigative study of AAV2/8 vector gene therapy for Fabry disease

Orphanet Journal of Rare Diseases, 2023
Background Fabry disease (FD) is a progressive multisystemic disease characterized by a lysosomal enzyme deficiency. A lack of α-galactosidase A (α-Gal A) activity results in the progressive systemic accumulation of its substrates, including ...
Mulan Deng, Hongyu Zhou, Shaomei He, Haoheng Qiu, Yanping Wang, April Yuanyi Zhao, Yunping Mu, Fanghong Li, Allan Zijian Zhao   +8 more
doaj   +1 more source

Inhibition of Vero Cell Cytotoxic Activity in Escherichia coli O157:H7 Lysates by Globotriaosylceramide, Gb3, from Bovine Milk

Bioscience, Biotechnology, and Biochemistry, 2001
In order to clarify the presence and verotoxin (VT) inhibitory activity of globotriaosylceramide (Gb3) in bovine milk, we analyzed neutral glycosphingolipids (GSLs) from bovine milk and investigated the inhibitory effect of bovine milk Gb3 on the cytotoxicity of VT2.
WATARAI, Shinobu, Tana, INOUE, Kaoru, KUSHI, Yasunori, ISOGAI, Emiko, YOKOTA, Kenji, NAKA, Katsumi, OGUMA, Keiji, KODAMA, Hiroshi   +8 more
openaire   +2 more sources

Could Fabry Disease Cause Giant Coronary Aneurysms in a 7‐Month‐Old Infant: A Case Report

Clinical Case Reports, Volume 14, Issue 7, July 2026.
ABSTRACT Fabry disease is a rare X‐linked lysosomal storage disorder that can affect multiple organs. Cardiac involvement, one of its significant manifestations, can begin in childhood and is more prevalent in males, with severity increasing with age and disease progression.
Reza Shabanian, Minoo Dadkhah, Rana Doroudian, Mehrnaz Adib, Sahar Naderi, Khashayar Ashkboos   +5 more
wiley   +1 more source