Results 41 to 50 of about 3,830 (166)

Binding of Pk-Trisaccharide Analogs of Globotriaosylceramide to Shiga Toxin Variants [PDF]

Infection and Immunity, 2013
ABSTRACT The two major forms of Shiga toxin, Stx1 and Stx2, use the glycolipid globotriaosylceramide (Gb3) as their cellular receptor. Stx1 primarily recognizes the Pk-trisaccharide portion and has three Pk binding sites per B monomer. The Stx2a subtype requires glycolipid residues in addition to Pk.
Hailemichael O, Yosief, Suri S, Iyer, Alison A, Weiss   +2 more
openaire   +2 more sources

Neutral glycosphingolipids in human blood: a precise mass spectrometry analysis with special reference to lipoprotein-associated Shiga toxin receptors[S]

Journal of Lipid Research, 2010
Shiga toxin (Stx)-producing Escherichia coli are the leading cause of hemorrhagic colitis and life-threatening extraintestinal complications in humans. Stx1 and Stx2 are transferred by yet to be delineated mechanisms from the intestine to the circulation
Christian H. Schweppe, Petra Hoffmann, Jerzy-Roch Nofer, Gottfried Pohlentz, Michael Mormann, Helge Karch, Alexander W. Friedrich, Johannes Müthing   +7 more
doaj   +1 more source

The effects of globotriaosylceramide tail saturation level on bilayer phases

Soft Matter, 2015
The globotriaosylceramide acyl chains from one leaflet interdigitate into the opposing leaflet and lead to significant bilayer rigidification and immobilisation of the lipid tails. Globotriaosylceramide with saturated acyl chains can form a highly ordered, relatively immobile phase which is resistant to bending.
Weria Pezeshkian, Vitaly V. Chaban, Ludger Johannes, Julian Shillcock, John H. Ipsen, Himanshu Khandelia   +5 more
openaire   +5 more sources

Unexpectedly high renal pathological scores of two female siblings with Fabry disease presenting with urinary mulberry cells without microalbuminuria

Molecular Genetics and Metabolism Reports, 2022
We describe the cases of 47- and 45-year-old sisters who were diagnosed with Fabry disease by genomic analysis. Although the only abnormal finding was the presence of mulberry cells in their urinary sediment, the renal pathological scores, which were ...
Natsuo Yamada, Hirofumi Sakuma, Mitsuru Yanai, Ayana Suzuki, Keisuke Maruyama, Motoki Matsuki, Naoki Nakagawa   +6 more
doaj   +1 more source

Structural Basis of Shiga Toxin Neutralization by Human Antibodies Targeting Canonical Receptor‐Binding and Non‐Canonical Assembly Sites

Advanced Science, EarlyView.
Cryo‐electron microscopy structures reveal two distinct neutralization mechanisms of fully human monoclonal antibodies targeting the Shiga toxin 1a B subunit. RDS059 occludes the Gb3 receptor‐binding site, whereas RDS045 engages a lateral quaternary epitope to disrupt holotoxin assembly.
Jianting Ke, Xin Li, Xiaoli Wang, Zhaoxia Huang, Shaojie Xue, Yuyang Liu, Jing Pan, Guowei Hu, Yayu Wang, Cheng Wang, Shoudeng Chen   +10 more
wiley   +1 more source

Epithelial-Mesenchymal Transition in Kidney Tubular Epithelial Cells Induced by Globotriaosylsphingosine and Globotriaosylceramide. [PDF]

PLoS ONE, 2015
Fabry disease is a lysosomal storage disorder caused by deficiency of alpha-galactosidase A (α-gal A), which results in the deposition of globotriaosylceramide (Gb3) in the vascular endothelium.
Yeo Jin Jeon, Namhee Jung, Joo-Won Park, Hae-Young Park, Sung-Chul Jung   +4 more
doaj   +1 more source

Does administration of hydroxychloroquine/amiodarone accelerate accumulation of globotriaosylceramide and globotriaosylsphingosine in Fabry mice?

Molecular Genetics and Metabolism Reports, 2021
Drug-induced lysosomal storage disease (DILSD) caused by cationic amphiphilic drugs (CADs), which exhibits toxic manifestations and pathological findings mimicking Fabry disease (α-galactosidase A deficiency), has attracted the interests of clinicians ...
Takahiro Tsukimura, Tomoko Shiga, Koki Saito, Yasuhiro Ogawa, Hitoshi Sakuraba, Tadayasu Togawa   +5 more
doaj   +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

Characterization of small fiber pathology in a mouse model of Fabry disease

eLife, 2018
Fabry disease (FD) is a life-threatening X-linked lysosomal storage disorder caused by α-galactosidase A (α-GAL) deficiency. Small fiber pathology and pain are major FD symptoms of unknown pathophysiology.
Lukas Hofmann, Dorothea Hose, Anne Grießhammer, Robert Blum, Frank Döring, Sulayman Dib-Hajj, Stephen Waxman, Claudia Sommer, Erhard Wischmeyer, Nurcan Üçeyler   +9 more
doaj   +1 more source

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