Altered dynamics of a lipid raft associated protein in a kidney model of Fabry disease
A Labilloy, RT Youker, JR Bruns, I Kukic… - Molecular genetics and …, 2014 - Elsevier
Accumulation of globotriaosylceramide (Gb3) and other neutral glycosphingolipids with
galactosyl residues is the hallmark of Fabry disease, a lysosomal storage disorder caused
by deficiency of the enzyme alpha-galactosidase A (α-gal A). These lipids are incorporated
into the plasma membrane and intracellular membranes, with a preference for lipid rafts.
Disruption of raft mediated cell processes is implicated in the pathogenesis of several
human diseases, but little is known about the effects of the accumulation of …
galactosyl residues is the hallmark of Fabry disease, a lysosomal storage disorder caused
by deficiency of the enzyme alpha-galactosidase A (α-gal A). These lipids are incorporated
into the plasma membrane and intracellular membranes, with a preference for lipid rafts.
Disruption of raft mediated cell processes is implicated in the pathogenesis of several
human diseases, but little is known about the effects of the accumulation of …