Chemically synthesized Gb3 glycosphingolipids: tools to access their function in lipid membranes
Sign Up to like & getrecommendations! Published in 2020 at "European Biophysics Journal"
DOI: 10.1007/s00249-020-01461-w
Abstract: Gb3 glycosphingolipids are the specific receptors for bacterial Shiga toxin. Whereas the trisaccharidic head group of Gb3 defines the specificity of Shiga toxin binding, the lipophilic part composed of sphingosine and different fatty acids is… read more here.
Keywords: gb3; gb3 glycosphingolipids; microscopy; fatty acid ... See more keywords
Globotriaosylsphingosine (Lyso-Gb3) as a biomarker for cardiac variant (N215S) Fabry disease
Sign Up to like & getrecommendations! Published in 2017 at "Journal of Inherited Metabolic Disease"
DOI: 10.1007/s10545-017-0127-2
Abstract: Fabry disease (FD) is a multi-systemic X-linked lysosomal disorder caused by the deficient activity of α-galactosidase-A enzyme, which leads to accumulation of glycosphingolipids in various body tissues. The N215S mutation is a known variant of… read more here.
Keywords: cardiac variant; lyso gb3; disease; gb3 ... See more keywords
Renal globotriaosylceramide facilitates tubular albumin absorption and its inhibition protects against acute kidney injury.
Sign Up to like & getrecommendations! Published in 2019 at "Kidney international"
DOI: 10.1016/j.kint.2019.02.010
Abstract: To elucidate the physiologic function of renal globotriaosylceramide (Gb3/CD77), which up-to-date has been associated exclusively with Shiga toxin binding, we have analyzed renal function in Gb3-deficient mice. Gb3 synthase KO (Gb3S-/-) mice displayed an increased… read more here.
Keywords: mice; gb3; kidney injury; renal globotriaosylceramide ... See more keywords
Correlation of Lyso-Gb3 levels in dried blood spots and sera from patients with classic and Later-Onset Fabry disease.
Sign Up to like & getrecommendations! Published in 2017 at "Molecular genetics and metabolism"
DOI: 10.1016/j.ymgme.2017.06.006
Abstract: BACKGROUND Fabry disease (FD), an X-linked lysosomal storage disorder, results from the deficient activity of α-galactosidase A (α-Gal A) and the accumulation of its substrates, globotriaosylceramide (Gb3) and its deacylated derivative, globotriaosyl-sphingosine (Lyso-Gb3). Here, we… read more here.
Keywords: gb3 levels; classic later; gb3; lyso gb3 ... See more keywords
Evaluation of long-term effects by ERT for Fabry disease biochemical and EM pictures
Sign Up to like & getrecommendations! Published in 2019 at "Molecular Genetics and Metabolism"
DOI: 10.1016/j.ymgme.2018.12.182
Abstract: Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of alpha-gal A, leading to the progressive accumulation of sphingolipids. Enzyme replacement therapy (ERT) is the most common therapy at present which has… read more here.
Keywords: fabry disease; gb3; biopsy; microscopy ... See more keywords
Does administration of hydroxychloroquine/amiodarone accelerate accumulation of globotriaosylceramide and globotriaosylsphingosine in Fabry mice?
Sign Up to like & getrecommendations! Published in 2021 at "Molecular Genetics and Metabolism Reports"
DOI: 10.1016/j.ymgmr.2021.100773
Abstract: 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 and pathologists. Although the affected… read more here.
Keywords: accumulation; gb3; hydroxychloroquine amiodarone; fabry mice ... See more keywords
Separation and Analysis of Lactosylceramide, Galabiosylceramide, and Globotriaosylceramide by LC-MS/MS in Urine of Fabry Disease Patients.
Sign Up to like & getrecommendations! Published in 2017 at "Analytical chemistry"
DOI: 10.1021/acs.analchem.7b03609
Abstract: Fabry disease is an X-linked lysosomal storage disorder caused by α-galactosidase A (α-GAL A) deficiency. This enzyme contributes to the cellular recycling of glycosphingolipids such as galabiosylceramide (Ga2), globotriaosylceramide (Gb3), and globotriaosylsphingosine (lyso-Gb3) by hydrolyzing… read more here.
Keywords: disease patients; gb3; analysis; fabry disease ... See more keywords
Differential recognition of lipid domains by two Gb3-binding lectins
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DOI: 10.1038/s41598-020-66522-8
Abstract: The two lectins LecA from Pseudomonas aeruginosa and the B-subunit of Shiga toxin from Shigella dysenteriae (StxB) share the glycosphingolipid globotriaosylceramide (Gb3) as receptor. Counterintuitively, we found that LecA and StxB segregated into different domains… read more here.
Keywords: leca stxb; gb3; recognition; membrane ... See more keywords
A phase II, multicenter, open-label trial to evaluate the safety and efficacy of ISU303 (Agalsidase beta) in patients with Fabry disease
Sign Up to like & getrecommendations! Published in 2022 at "Medicine"
DOI: 10.1097/md.0000000000030345
Abstract: Background: Fabry disease (FD) is caused by a deficiency in the activity of the lysosomal enzyme, α-galactosidase A (α-Gal A), which leads to globotriaosylceramide (Gb3) deposition in multiple tissues. The current management of FD is… read more here.
Keywords: safety; gb3; plasma urine; efficacy ... See more keywords
Pulmonary involvement in Fabry disease: effect of plasma globotriaosylsphingosine and time to initiation of enzyme replacement therapy
Sign Up to like & getrecommendations! Published in 2018 at "BMJ Open Respiratory Research"
DOI: 10.1136/bmjresp-2018-000277
Abstract: Introduction Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by mutations of GLA gene leading to reduced α-galactosidase activity and resulting in a progressive accumulation of globotriaosylceramide (Gb3) and its deacylated derivative, globotriaosyl-sphingosine… read more here.
Keywords: plasma; gb3; initiation; lyso gb3 ... See more keywords
Repurposing of tamoxifen ameliorates CLN3 and CLN7 disease phenotype
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DOI: 10.15252/emmm.202013742
Abstract: Batten diseases (BDs) are a group of lysosomal storage disorders characterized by seizure, visual loss, and cognitive and motor deterioration. We discovered increased levels of globotriaosylceramide (Gb3) in cellular and murine models of CLN3 and… read more here.
Keywords: tamoxifen ameliorates; gb3; disease; tamoxifen ... See more keywords