Globotriaosylceramides (hydroxy) (porcine)

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Globotriaosycleramides are glycosphingolipids found in mammalian cell membranes that are synthesized from lactosylceramides . They act as receptors for Shiga and Shiga-like toxins in vitro and in vivo. Globotriaosylceramides accumulate in endothelial cells, pericytes, vascular smooth muscle cells, renal epithelial cells, dorsal ganglia neuronal cells, and myocardial cells in patients with Fabry disease, a lysosomal storage disorder characterized by a deficiency in the enzyme α-galactosidase A. Globotriaosylceramides act as natural resistance factors to HIV infection, interacting with HIV gp120 to prevent its interaction with chemokine co-receptors and subsequent fusion of HIV to host cell membranes. This product contains a mixture of hydroxy fatty acid-containing globotriaosylceramides isolated from porcine red blood cells (RBCs).