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RIPK3 Contributes to Lyso-Gb3-Induced Podocyte Death

open access: yesCells, 2021
Fabry disease is a lysosomal storage disease with an X-linked heritage caused by absent or decreased activity of lysosomal enzymes named alpha-galactosidase A (α-gal A).
Jong-Seok Moon   +2 more
exaly   +2 more sources
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Gb3-Coated Bovine Milk Exosomes as a Practical Neutralizer for Shiga Toxin

ACS Applied Bio Materials, 2023
Shiga toxin (Stx) is associated with foodborne infections of some Shigella spp. and Shiga toxin-producing Escherichia coli (STEC), leading to life-threatening hemolytic uremic syndrome (HUS).
Zhimeng Wu, Zhifang Zhou, Haofei Hong
exaly   +2 more sources

The use and performance of lyso-Gb3 for the diagnosis and monitoring of Fabry disease: A systematic literature review

open access: yesMolecular Genetics and Metabolism
BACKGROUND Fabry disease (FD) is a rare, X-linked lysosomal storage disorder in which a lack of alpha-galactosidase (α-Gal A) enzyme activity leads to intracellular accumulation of deacylated globotriaosylceramide (Gb3) and globotriaosylsphingosine (lyso-
Karen Tylee   +2 more
exaly   +2 more sources

A Rapid and Simple UHPLC-MS/MS Method for Quantification of Plasma Globotriaosylsphingosine (lyso-Gb3)

open access: yesMolecules, 2021
Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by α-galactosidase A gene (GLA) mutations, resulting in loss of activity of the lysosomal hydrolase, α-galactosidase A (α-Gal A).
Alessandro Perrone   +2 more
exaly   +2 more sources

One of the two N-glycans on the human Gb3/CD77 synthase is essential for its activity and allosterically regulates its function.

Biochemical and Biophysical Research Communications - BBRC, 2022
N-glycosylation is a posttranslational modification that influences many protein properties, such as bioactivity, folding or solubility. The same principles apply to key enzymes in glycosylation pathways, including glycosyltransferases, that also ...
Krzysztof Mikołajczyk   +4 more
semanticscholar   +1 more source

Two Paralogous Gb3/CD77 Synthases in Birds Show Different Preferences for Their Glycoprotein and Glycosphingolipid Substrates

open access: yesInternational Journal of Molecular Sciences, 2021
Most glycosyltransferases show remarkable gross and fine substrate specificity, which is reflected in the old one enzyme-one linkage paradigm. While human Gb3/CD77 synthase is a glycosyltransferase that synthesizes the Galα1→4Gal moiety mainly on ...
Edyta Majorczyk   +2 more
exaly   +2 more sources

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