Globotriaosylceramide Gb3 Influences Wound Healing and Scar Formation by Orchestrating Fibroblast Heterogeneity. [PDF]
Adv Sci (Weinh)Cutaneous fibroblast heterogeneity is mechanistically linked to wound repair outcomes and fibrotic progression, with glycosphingolipid metabolism emerging as a critical determinant of physiological fibroblast diversity.
Xie S +13 more
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Sex differences of urinary and kidney globotriaosylceramide and lyso-globotriaosylceramide in Fabry mice [PDF]
Journal of Lipid Research, 2011 The aim of our study was to measure globotriaosylceramide (Gb3) and lyso-Gb3 levels by tandem mass spectrometry in the urine and kidney in Fabry (gla knockout) mice and wild-type controls.
Brandon Durant +7 more
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Role of Globotriaosylceramide in Physiology and Pathology
Frontiers in Molecular Biosciences, 2022 At first glance, the biological function of globoside (Gb) clusters appears to be that of glycosphingolipid (GSL) receptors for bacterial toxins that mediate host-pathogen interaction.
Ana Beatriz Celi +6 more
doaj +4 more sources
Impact of migalastat therapy on corneal deposits in a female with Fabry disease: A case report. [PDF]
Mol Genet Metab RepFabry disease is a rare X-linked lysosomal storage disorder caused by alpha-galactosidase A deficiency, leading to globotriaosylceramide accumulation in multiple organs, including the eye, where corneal verticillata represents a typical sign.
Vitturi N +5 more
europepmc +2 more sources
A Rare Case of Fabry Disease Combined With Idiopathic Multicentric Castleman Disease and Membranous Nephropathy [PDF]
Kidney MedThe present case documents a 17-year-old woman patient with nephrotic syndrome who was diagnosed with Fabry disease complicated by idiopathic multicentric Castleman disease (iMCD) and membranous nephropathy.
Oka M, Suzuki R, Akasaka T.
europepmc +2 more sources
In vitro effect of globotriaosylceramide on electron transport chain complexes and redox parameters [PDF]
Anais da Academia Brasileira de Ciências, 2019 : Fabry disease (FD) is an X-linked inherited disease and occurs due to mutations in GLA gene that encodes the α-galactosidase enzyme. Consequently, there is an accumulation of enzyme substrates, namely globotriaosylceramide (GB3).
RAFAELA M. ALVARIZ +4 more
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Carbon-13 nuclear magnetic resonance spectrometry of globotriaosylceramide
Journal of Lipid Research, 1982 Resonances in the carbon-13 natural abundance, proton-decoupled, 90.5 MHz nuclear magnetic resonance spectrum of globotriaosylceramide were assigned to specific carbon nuclei. The chemical shifts were rationalized in terms of the number of sugar residues,
H A Nunez, C C Sweeley
doaj +3 more sources
Cellular signaling pathway of Shiga toxin-induced ATP release. [PDF]
Front Cell Infect MicrobiolBackgroundShiga toxin (Stx) is the main virulence factor of enterohemorrhagic Escherichia coli, a food-borne pathogen that colonizes the intestine causing gastroenteritis and, in severe cases, hemolytic uremic syndrome.
Johansson K +4 more
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Molecular Genetics and Metabolism Reports, 2023 Fabry disease (FD) is an inherited disease caused by deficient α-galactosidase A activity that is characterized by the accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-Gb3).
Atsumi Taguchi +3 more
doaj +3 more sources
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 +5 more sources