Results 31 to 40 of about 31,524 (206)
Journal of Clinical Medicine, 2023 Background: Fabry disease cardiomyopathy (FDCM) has manifested some resistance to enzyme replacement therapy (ERT), particularly in its advanced phase. Recently, myocardial inflammation of autoimmune origin has been demonstrated in FDCM.
A. Frustaci +5 more
semanticscholar +1 more source
Assessing the role of glycosphingolipids in the phenotype severity of Fabry disease mouse model
Journal of Lipid Research, 2020 Fabry disease is caused by deficient activity of α-galactosidase A, an enzyme that hydrolyzes the terminal α-galactosyl moieties from glycolipids and glycoproteins, and subsequent accumulation of glycosphingolipids, mainly globotriaosylceramide (Gb3 ...
Siamak Jabbarzadeh-Tabrizi +6 more
doaj +1 more source
Nano-LC-MS/MS for Quantification of Lyso-Gb3 and Its Analogues Reveals a Useful Biomarker for Fabry Disease. [PDF]
PLoS ONE, 2015 Biomarkers useful for diagnosis and evaluation of treatment for patients with Fabry disease are urgently needed. Recently, plasma globotriaosylsphingosine (lyso-Gb3) and lyso-Gb3-related analogues have attracted attention as promising biomarkers of Fabry
Hideaki Sueoka +4 more
doaj +1 more source
Novel lectin-based chimeric antigen receptors target Gb3-positive tumour cells
Cellular and Molecular Life Sciences, 2022 The link between cancer and aberrant glycosylation has recently become evident. Glycans and their altered forms, known as tumour-associated carbohydrate antigens (TACAs), are diverse, complex and difficult to target therapeutically. Lectins are naturally
A. V. Meléndez +10 more
semanticscholar +1 more source
Lyso-Gb3 associates with adverse long-term outcome in patients with Fabry disease
Journal of Medical Genetics, 2021 Background Fabry disease (FD) is a rare X-linked lysosomal storage disease caused by mutations in the α-galactosidase A gene (GLA) leading to deficiency of α-galactosidase A and ultimately in progressive glycosphingolipid accumulation, especially ...
A. Nowak +4 more
semanticscholar +1 more source
Gb3/cd77 Is a Predictive Marker and Promising Therapeutic Target for Head and Neck Cancer
Biomedicines, 2022 Head and neck squamous cell carcinoma is the sixth leading cancer in the world. This cancer is difficult to treat and is characterized by recurrences that are often fatal.
L. García‐Hevia +6 more
semanticscholar +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
Frontiers in Cellular and Infection Microbiology, 2020 Shiga toxin is the main virulence factor of non-invasive enterohemorrhagic Escherichia coli strains capable of causing hemolytic uremic syndrome. Our group has previously shown that the toxin can reach the kidney within microvesicles where it is taken up
Karl Johansson +6 more
doaj +1 more source
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
Journal of Pharmacy and Science, 2022 Globotriaosylceramide (Gb3 or CD77) is a tumor-associated carbohydrate antigen implicated in several types of cancer that serves as a potential cancer marker for developing target-specific diagnosis and therapy.
Zhicheng Liu +8 more
semanticscholar +1 more source