Results 51 to 60 of about 3,638 (152)

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

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

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

Risk of Death in Heart Disease is Associated With Elevated Urinary Globotriaosylceramide [PDF]

Journal of the American Heart Association, 2014
Background Elevated urinary globotriaosylceramide (Gb 3 ) has been considered a hallmark of F abry disease, an X‐linked lysosomal disorder that is a risk factor for most types of heart disease.
Schiffmann, R., Forni, S., Swift, C., Brignol, N., Wu, X., Lockhart, D., Blankenship, D., Wang, X., Grayburn, P., Taylor, M., Lowes, B., Fuller, M., Benjamin, E., Sweetman, L.   +13 more
openaire   +3 more sources

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

Septins in the Middle—Makers and Breakers of Membrane Contact Sites

Journal of Neurochemistry, Volume 170, Issue 6, June 2026.
Septins are a family of GTP‐binding proteins that assemble into heteromeric oligomers and polymers, associating with specific membrane domains and organelles according to their subunit composition. Growing evidence places septins at membrane contact sites (MCS) — key hubs for intracellular communication that mediate exchange of ions, lipids, and ...
TrishaJean J. Holt, Elias T. Spiliotis
wiley   +1 more source

A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis [PDF]

Biochemical Journal, 2013
Fabry disease is a lysosomal storage disorder in which neutral glycosphingolipids, predominantly Gb3 (globotriaosylceramide), accumulate due to deficient α-Gal A (α-galactosidase A) activity. The GLAko (α-Gal A-knockout) mouse has been used as a model for Fabry disease, but it does not have any symptomatic abnormalities.
Atsumi, Taguchi, Hiroki, Maruyama, Masaaki, Nameta, Tadashi, Yamamoto, Junichiro, Matsuda, Ashok B, Kulkarni, Hidekatsu, Yoshioka, Satoshi, Ishii   +7 more
openaire   +2 more sources

p.R220L Is a Likely Pathogenic Novel GLA Gene Mutation Responsible for Fabry Disease

Anatolian Journal of Cardiology, 2022
Fabry disease is a progressive and rare storage disease that occurs due to low or complete deficiency of lysosomal alpha galactosidase-A (α-GLA) enzyme activity.
Hasan Ali Barman, Adem Atıcı, Serhan Özyıldırım, Serdar Ceylaner, Memduh Dursun, Sait Mesut Doğan   +5 more
doaj   +1 more source

Accumulation of Globotriaosylceramide in Podocytes in Fabry Nephropathy Is Associated with Progressive Podocyte Loss [PDF]

Journal of the American Society of Nephrology, 2020
Significance Statement In males with classic Fabry disease, the processes leading to the frequent outcome of ESKD are poorly understood. Mutations in the gene encoding α-galactosidase A leads to globotriaosylceramide accumulation in various cell types; in podocytes, this accumulation progresses with age.
Najafian, Behzad, Tøndel, Camilla, Svarstad, Einar, Gubler, Marie-Claire, Oliveira, Joao-Paulo, Mauer, Michael   +5 more
openaire   +4 more sources