Results 21 to 30 of about 30,590 (168)
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 +5 more
doaj +1 more source
Lyso-Gb3 activates Notch1 in human podocytes [PDF]
Human Molecular Genetics, 2015 Podocyte injury is an early feature of Fabry nephropathy, but the molecular mechanisms of podocyte injury are poorly understood. Lyso-Gb3 accumulates in serum in Fabry disease and increases extracellular matrix synthesis in podocytes. We explored the contribution of Notch1 signaling, a mediator of podocyte injury, to lyso-Gb3-elicited responses in ...
Maria D, Sanchez-Niño +5 more
openaire +2 more sources
Skin globotriaosylceramide 3 deposits are specific to Fabry disease with classical mutations and associated with small fibre neuropathy. [PDF]
PLoS ONE, 2017 Fabry Disease (FD) is characterized by globotriaosylceramide-3 (Gb3) accumulation in several tissues and a small fibre neuropathy (SFN), however the underlying mechanisms are poorly known. This study aimed to: 1) ascertain the presence of Gb3 deposits in
Rocco Liguori +15 more
doaj +1 more source
Regulation of Gb3 Expression on Dendritic Cells [PDF]
Journal of Life Science, 2007 Infection with Shiga-like toxin (SLT)-producing Escherichia coli causes a spectrum of illnesses with high morbidity and mortality. Host mediators play an important role in the pathogenesis of SLT-I toxicity. We here investigated the effect of SLT-I on tumor necrosis factor-α (TNF-α) production, effect of TNF-α on glycolipid globotriaosyleramide (Gb3 ...
Suk-Hwan Lim +6 more
openaire +1 more source
Molecular Genetics and Metabolism Reports, 2021 In Fabry disease, accumulation of glycolipids, predominantly globotriaosylceramide (Gb3), affects the kidneys, and nephropathy is one of the important disorders that influence the disease severity and prognosis of patients.
Tomoko Shiga +4 more
doaj +1 more source
Skin Globotriaosylceramide 3 Load Is Increased in Men with Advanced Fabry Disease. [PDF]
PLoS ONE, 2016 The X-chromosomally linked life-limiting Fabry disease (FD) is associated with deposits of the sphingolipid globotriaosylceramide 3 (Gb3) in various tissues. Skin is easily accessible and may be used as an additional diagnostic and follow-up medium.
Nurcan Üçeyler +6 more
doaj +1 more source
Tumor-specific Hsp70 plasma membrane localization is enabled by the glycosphingolipid Gb3. [PDF]
PLoS ONE, 2008 Human tumors differ from normal tissues in their capacity to present Hsp70, the major stress-inducible member of the HSP70 family, on their plasma membrane.
Mathias Gehrmann +7 more
doaj +1 more source
Two Distinct Gb3/CD77 Signaling Pathways Leading to Apoptosis Are Triggered by Anti-Gb3/CD77 mAb and Verotoxin-1 [PDF]
Journal of Biological Chemistry, 2003 Globotriasosylceramide (Gb3), a neutral glycosphingolipid, is the B-cell differentiation antigen CD77 and acts as the receptor for most Shiga toxins, including verotoxin-1 (VT-1). We have shown that both anti-Gb3/CD77 mAb and VT-1 induce apoptosis in Burkitt's lymphoma cells.
Tétaud, Cécile +10 more
openaire +2 more sources
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 +4 more
doaj +1 more source
Entropy Drives the Formation of Salt Bridges in the Protein GB3 [PDF]
Angewandte Chemie International Edition, 2017 AbstractSalt bridges are very common in proteins. But what drives the formation of protein salt bridges is not clear. In this work, we determined the strength of four salt bridges in the protein GB3 by measuring the ΔpKa values of the basic residues that constitute the salt bridges with a highly accurate NMR titration method at different temperatures ...
Ning Zhang +6 more
openaire +2 more sources