Results 11 to 20 of about 3,683 (169)
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
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
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
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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
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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Fabry nephropathy before and after enzyme replacement therapy: important role of renal biopsy in patients with Fabry disease [PDF]
Kidney Research and Clinical Practice, 2021 Background In Fabry disease, the presence of globotriaosylceramide (GL3) deposits in various kidney cells leads to progressive renal dysfunction. However, kidney biopsy studies in patients with Fabry disease are limited.
Il Young Kim +2 more
doaj +1 more source
Cells, 2022 Fabry disease (FD) is a rare life-threatening disorder caused by deficiency of the alpha-galactosidase A (GLA) enzyme with a characteristic pain phenotype.
Marlene Spitzel +8 more
doaj +1 more source
Accumulation of globotriaosylceramide in a case of leiomyosarcoma [PDF]
Biochemical Journal, 1986 Analysis of the glycosphingolipid composition in one case of uterine leiomyosarcoma metastasized to the liver showed an accumulation of globotriaosylceramide as compared with normal liver and uterus from which the tumour originated. The structure and the amount of glycosphingolipids were established by using specific glycosidases, permethylation ...
S C, Li +3 more
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Inhibition of tumor angiogenesis by globotriaosylceramide immunotargeting [PDF]
OncoImmunology, 2013 Current antiangiogenic immunotherapeutic strategies mainly focus on the blockade of circulating cytokines or receptors that are overexpressed by endothelial cells. We proposed globotriaosylceramide (Gb3) as a viable alternative target for antiangiogenic therapies. In this setting, we developed an anti-Gb3 antibody and validated its therapeutic efficacy
Birklé, S. +9 more
openaire +4 more sources