Globotriaosylceramide induces oxidative stress and up-regulates cell adhesion molecule expression in Fabry disease endothelial cells
JS Shen, XL Meng, DF Moore, JM Quirk… - Molecular genetics and …, 2008 - Elsevier
Fabry disease, an X-linked systemic vasculopathy, is caused by a deficiency of α-
galactosidase A resulting in globotriaosylceramide (Gb3) storage in cells. The pathogenic
role of Gb3 in the disease is not known. Based on previous work, we tested the hypothesis
that accumulation of Gb3 in the vascular endothelium of Fabry disease is associated with
increased production of reactive oxygen species (ROS) and increased expression of cell
adhesion molecules. Gb3-loading resulted in increased intracellular ROS production in …
galactosidase A resulting in globotriaosylceramide (Gb3) storage in cells. The pathogenic
role of Gb3 in the disease is not known. Based on previous work, we tested the hypothesis
that accumulation of Gb3 in the vascular endothelium of Fabry disease is associated with
increased production of reactive oxygen species (ROS) and increased expression of cell
adhesion molecules. Gb3-loading resulted in increased intracellular ROS production in …