Results 91 to 100 of about 5,162 (179)

MiRNA Let-7a and Let-7d Are Induced by Globotriaosylceramide via NF-kB Activation in Fabry Disease

, 2021
Background: Fabry disease is a hereditary genetic defect resulting in reduced activity of the enzyme α-galactosidase-A and the accumulation of globotriaosylceramide (Gb3) in body fluids and cells. Gb3 accumulation was especially reported for the vascular
Johann Wojta, Max Lenz, Manuel Salzmann, Patrick Haider, Bruno K. Podesser, Walter S. Speidl, Gere Sunder-Plassmann, Philipp J. Hohensinner, Nadine Maier, Constantin Gatterer, Senta Graf, Christoph Kaun   +11 more
core   +1 more source

Effects of Catfish Egg Lectin on Cancer Cells Differ According to the Globotriaosylceramide Species They Express. [PDF]

Int J Mol Sci
Silurus asotus (Amur catfish) egg lectin (SAL) inhibits cell proliferation and enhances the effects of anticancer drugs by binding to globotriaosylceramide (Gb3) on the cell surface. Gb3 expression is typically increased in seminomas.
Sugawara S, Kikuchi K, Tatsuta T, Fujimura T, Hosono M.   +4 more
europepmc   +2 more sources

Gb3‐cSrc complex in glycosphingolipid‐enriched microdomains contributes to the expression of p53 mutant protein and cancer drug resistance via β‐catenin–activated RNA methylation

FASEB BioAdvances, 2020
Glucosylceramide synthase (GCS) is a key enzyme catalyzing ceramide glycosylation to generate glucosylceramide (GlcCer), which in turn serves as the precursor for cells to produce glycosphingolipids (GSLs).
Kartik R. Roy, Mohammad B. Uddin, Sagor C. Roy, Ronald A. Hill, John Marshall, Yu‐Teh Li, Jean Christopher Chamcheu, Hua Lu, Yong‐Yu Liu   +8 more
doaj   +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, Beth Binnington, Darinka Sakac, Yulia Katsman, Stephanie Ramkumar, Jean Gariepy, Minji Kim, Donald R. Branch, Clifford Lingwood   +8 more
doaj   +1 more source

Imbalanced Production of Reactive Oxygen Species and Mitochondrial Antioxidant SOD2 in Fabry Disease-Specific Human Induced Pluripotent Stem Cell-Differentiated Vascular Endothelial Cells

Cell Transplantation, 2017
Fabry disease (FD) is an X-linked inherited lysosomal storage disease caused by α-galactosidase A (GLA) deficiency. Progressive intracellular accumulation of globotriaosylceramide (Gb3) is considered to be pathogenically responsible for the phenotype ...
Wei-Lien Tseng, Shih-Jie Chou, Huai-Chih Chiang, Mong-Lien Wang, Chian-Shiu Chien, Kuan-Hsuan Chen, Hsin-Bang Leu, Chien-Ying Wang, Yuh-Lih Chang, Yung-Yang Liu, Yuh-Jyh Jong, Shinn-Zong Lin, Shih-Hwa Chiou, Shing-Jong Lin, Wen-Chung Yu   +14 more
doaj   +1 more source

Electrocardiographic Changes and Arrhythmia in Fabry Disease

Frontiers in Cardiovascular Medicine, 2016
Fabry disease is an X-chromosome-linked lysosomal storage disease characterized by a deficient activity or, in most males, absence of the enzyme alpha-galactosidase A (a-Gal A) leading to systemic, primary lysosomal accumulation of globotriaosylceramide (
Mehdi eNamdar
doaj   +1 more source

Gb3 検出に利用する抗Gb3 抗体（1A4）の有用性の検討 [PDF]

, 2018
globotriaosylceramide（gb3）is the most important cell surface ligand of the rhamnose-binding lectins such as Sirulus asotus lectin（SaL）．SaL recognizes and binds to gb3 expressing tumor cells such as Burkitts lymphoma raji cells and induces them to the g0 ...
鷹觜 祥子, 佐藤 信, 細野 雅祐, 立田 岳生, 大山 力, 伊藤 淳, 本田 捷太, 菅原 栄紀   +7 more
core  

Biochemical and clinical analysis of accumulated glycolipids in symptomatic heterozygotes of angiokeratoma corporis diffusum (Fabry's disease) in comparison with hemizygotes.

Journal of Lipid Research, 1990
Angiokeratoma corporis diffusum (Fabry's disease) is an X-linked disorder of glycosphingolipid catabolism. Heterozygous females, although usually asymptomatic, are occasionally as severely afflicted as hemizygous males; recently we identified a ...
I Hozumi, M Nishizawa, T Ariga, T Miyatake   +3 more
doaj   +1 more source

Screening for Fabry Disease (FD) through immunohistochemistry for globotriaosylceramide (Gb3) in patients with segmental and focal glomerular sclerosis lesions and negative immunofluorescence for deposits in renal needle biopsies

, 2022
Contexto: A doença de Fabry (DF) é um distúrbio de armazenamento lisossomal ligado a X, causado pela atividade deficiente da enzima α-galactosidase A, resultando no acúmulo de glicoesfingolipídios, particularmente a globotriaosilceramida (Gb3) e ...
Zogheib, Rodrigo José Pizzello [UNESP]
core  

A novel rapid MALDI-TOF-MS-based method for measuring urinary globotriaosylceramide in Fabry patients [PDF]

, 2016
Fabry disease is an X-linked lysosomal storage disorder caused by deficiency of α-galactosidase A, resulting in the accumulation of glycosphingolipids in various organs.
Douglas G. Ward, Geberhiwot, Tarekegn, Derralynn A. Hughes, Hughes, Derralynn A, Alharbi, Fahad J, Tarekegn Geberhiwot, Fahad J. Alharbi, Ward, Douglas G; id_orcid   +7 more
core   +1 more source