Results 101 to 110 of about 31,524 (206)
Neurobiology of DiseaseFabry disease (FD) is an X-linked lysosomal storage disorder caused by pathogenic variants in the GLA gene that reduce α-galactosidase A activity and lead to progressive accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids.
Marco Salluzzo +13 more
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Angewandte Chemie, 2019 The receptor lipid Gb3 is responsible for the specific internalization of Shiga toxin (STx) into cells. The head group of Gb3 defines the specificity of STx binding, and the backbone with different fatty acids is expected to influence its localization ...
Jeremias Sibold +5 more
semanticscholar +1 more source
Verotoxin A Subunit Protects Lymphocytes and T Cell Lines against X4 HIV Infection in Vitro
Toxins, 2012 Our previous genetic, pharmacological and analogue protection studies identified the glycosphingolipid, Gb3 (globotriaosylceramide, Pk blood group antigen) as a natural resistance factor for HIV infection. Gb3 is a B cell marker (CD77), but a fraction of
Pei Lin Shi +8 more
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In vitro effect of globotriaosylceramide on electron transport chain complexes and redox parameters
Anais da Academia Brasileira de Ciências: 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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Automated quantification of skin Gb3 load and white matter lesion assessment in Fabry disease
Orphanet Journal of Rare DiseasesBackground Fabry disease (FD) is an X-linked lysosomal storage disorder characterized by cellular accumulation of globotriaosylceramide (Gb3). Methods We investigated dermal Gb3 load as a potential surrogate marker for systemic FD manifestations by ...
Catharina Pfister +10 more
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Journal of Lipid Research, 1992 The activity of alpha-galactosyltransferase in cultured rat pheochromocytoma subcloned (PC12h) cells was examined using Gb3 as the acceptor for the galactose from UDP-galactose.
S Pal, M Saito, T Ariga, RK Yu
doaj +1 more source
International Journal of Molecular Sciences, 2019 Glycosphingolipids are known to play a role in developing and maintaining the integrity of various organs and tissues. Among glycosphingolipids, there are several reports on the involvement of gangliosides in bone metabolism.
K. Hamamura +12 more
semanticscholar +1 more source
Glykosphingolipide Gb3 und iGb3 [PDF]
Der Pathologe, 2008 S. Porubsky +6 more
openaire +1 more source
Physical Chemistry, Chemical Physics - PCCP, 2019 We studied the influence of globotriaosylceramide (Gb3) lipid molecules on the properties of phospholipid membranes composed of a liquid ordered (lo)/liquid disordered (ld) phase separated 1-palmitoyl-2-oleoyl-sn-glycero-3-phosphocholine (POPC)/N ...
Mathias Bosse +8 more
semanticscholar +1 more source
Regulation of Globotriaosylceramide (Gb3)‐Mediated Signal Transduction by Rhamnose‐Binding Lectin
ChemInform, 2007 AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 200 leading journals. To access a ChemInform Abstract, please click on HTML or PDF.
Kazuo, Nitta +3 more
openaire +3 more sources