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Higher apoptotic state in Fabry disease peripheral blood mononuclear cells: effect of globotriaosylceramide
(Elsevier Inc, 2011-11)
Fabry disease is an X-linked lysosomal storage disorder (LSD) due to deficiency of the enzyme α-galactosidase A, resulting in intracellular deposition of globotriaosylceramide (Gb3). Accumulation of Gb3 is probably related ...
A glucosylceramide synthase inhibitor prevents the cytotoxic effects of Shiga toxin-2 on human renal tubular epithelial cells
(Bentham Science Publishers, 2008-12)
Shiga toxin-2 binds to the globotriaosyl-ceramide receptor on the plasma membrane of target cells. The highlevel expression of this receptor in renal epithelial cells may account, at least in part, for acute renal failure ...
Fabry disease: Treatment and diagnosis
(John Wiley & Sons Inc, 2009-11)
Fabry disease is an X-linked lysosomal disorder that results from a deficiency of the lysosomal enzyme α-galactosidase A leading to accumulation of glycolipids, mainly globotriaosylceramide in the cells from different ...
Intracerebroventricular Shiga toxin 2 increases the expression of its receptor globotriaosylceramide and causes dendritic abnormalities
(Elsevier Science, 2010-05)
Neurological damage caused by intoxication with Shiga toxin (Stx) from enterohemorrhagic Escherichia coli is the most unrepairable and untreatable outcome of Hemolytic Uremic Syndrome, and occurs in 30% of affected infants. ...
Enzyme replacement therapy for Anderson-Fabry disease.
(2013-04-03)
Anderson-Fabry disease is an X-linked defect of glycosphingolipid metabolism. Progressive renal insufficiency is a major source of morbidity, additional complications result from cardio- and cerebro-vascular involvement. ...
Enzyme replacement therapy for Anderson-Fabry disease.
(2013-04-03)
Anderson-Fabry disease is an X-linked defect of glycosphingolipid metabolism. Progressive renal insufficiency is a major source of morbidity, additional complications result from cardio- and cerebro-vascular involvement. ...