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Tu-P7:239 Cellular and tissue distribution of globotriaosylceramide (GB3) in fabry disease (FD)
Hassan Askari +4 more
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Pablo N. De Francesco +3 more
semanticscholar +3 more sources
Efficient generation of useful monoclonal antibodies (mAbs) with high performance in cancer therapeutics has been expected. Generation of mAbs reactive with globotriaosylceramide (Gb3/CD77) was compared between A/J mice and Gb3/CD77 synthase-deficient (A4GalT-knockout) mice by immunizing Gb3-liposome. Specificity and functions of established antibodies
Yuji Kondo +7 more
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Current Protocols in Human Genetics, 2017
AbstractFabry disease is a complex, panethnic lysosomal storage disorder. It is characterized by the accumulation of glycosphingolipids in tissues, organs, the vascular endothelium, and biological fluids. The reported incidence in different populations is quite variable, ranging from 1:1400 to 1:117,000.
Christiane, Auray-Blais +3 more
openaire +3 more sources
AbstractFabry disease is a complex, panethnic lysosomal storage disorder. It is characterized by the accumulation of glycosphingolipids in tissues, organs, the vascular endothelium, and biological fluids. The reported incidence in different populations is quite variable, ranging from 1:1400 to 1:117,000.
Christiane, Auray-Blais +3 more
openaire +3 more sources
Analytica Chimica Acta, 2018
Fabry disease is an X-linked lysosomal storage disorder with marked variability in the phenotype and genotype. Glycosphingolipids such as globotriaosylceramide (Gb3) isoforms/analogs, globotriaosylsphingosine (lyso-Gb3) and analogs, and galabiosylceramide (Ga2) isoforms/analogs may accumulate in biological fluids and different organs.
Amanda, Toupin +8 more
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Fabry disease is an X-linked lysosomal storage disorder with marked variability in the phenotype and genotype. Glycosphingolipids such as globotriaosylceramide (Gb3) isoforms/analogs, globotriaosylsphingosine (lyso-Gb3) and analogs, and galabiosylceramide (Ga2) isoforms/analogs may accumulate in biological fluids and different organs.
Amanda, Toupin +8 more
openaire +3 more sources
Inefficient nuclear incorporation of foreign DNA remains a critical roadblock in the development of effective nonviral gene delivery systems. DNA delivered by traditional protocols remains within endosomal/lysosomal vesicles, or is rapidly degraded in the cytoplasm.
Linda M. Facchini, Clifford A. Lingwood
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Neutral glycosphingolipids (GSLs) are considered activation markers on human lymphocytes, which are fundamental for studying the immune system. For cattle, only a limited number of activation markers has yet been identified. We recently showed that Shiga toxin 1, known to use globotriaosylceramide (Gb(3) syn.
Christian Menge +5 more
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Lectin-Based Substrate Detection in Fabry Disease Using the Gb3-Binding Lectins StxB and LecA
International Journal of Molecular SciencesFabry disease, the second most common lysosomal storage disorder, is caused by a deficiency of α-galactosidase A (α-Gal A), which leads to an accumulation of glycosphingolipids (GSL), mainly globotriaosylceramide (also known as Gb3).
Serap Elçin-Guinot +7 more
semanticscholar +1 more source
The lipid globotriaosylceramide promotes germinal center B cell responses and antiviral immunity
ScienceInfluenza viruses escape immunity owing to rapid antigenic evolution, which requires vaccination strategies that allow for broadly protective antibody responses.
Pankaj Sharma +16 more
semanticscholar +1 more source

