Search results for "Ceramide"

Effects of enzyme replacement therapy with agalsidase alfa on glomerular filtration rate in patients with Fabry disease: preliminary data

2007

Progressive deposition of globotriaosylceramide results in severe complications involving the kidney, heart and brain in both hemizygous male and heterozygous female patients with Fabry disease. Analysis of renal data from FOS - the Fabry Outcome Survey - suggests that enzyme replacement therapy with agalsidase alfa can significantly improve renal function in patients with Fabry disease, at least in those with a mild decrease in glomerular filtration rate, and may also be able to slow down the natural decline in renal function in patients with a moderate reduction in glomerular filtration rate. Conclusion: Initial results from the large cohort of patients within FOS indicate that treatment …

A study of plasma and urinary sediment globotriaosylceramide levels in females with Fabry disease

2007

Immune Modulating Effects of NKT Cells in a Physiologically Low Dose Leishmania major Infection Model after αGalCer Analog PBS57 Stimulation

2014

Leishmaniasis is a parasitic infection affecting ∼12 million people worldwide, mostly in developing countries. Treatment options are limited and no effective vaccines exist to date. Natural Killer T (NKT) cells are a conserved innate-like lymphocyte population with immunomodulating effects in various settings. A number of reports state a role of NKT cells in different models of Leishmania infection. Here, we investigated the effect of NKT cells in a physiologically relevant, intradermal low dose infection model. After inoculation of 103 infectious-stage L. major, comparable numbers of skin-immigrating NKT cells in both susceptible BALB/c mice and resistant C57BL/6 mice were noted. Compared …

4812 - A0028 Gangliosides in the retina: a comparative inventory with brain and other ocular structures

2016

Purpose: Gangliosides (GG) make an extremely diverse family of glycosphingolipids particularly abundant in the brain and neural tissues. While it is known that alterations in GG metabolism are associated with visual defects, the precise biological roles of these compounds in the retina are still largely unknown. In this context, we performed an exhaustive inventory and thorough characterization of the human eye GG aiming to reveal specificities and common features of the retina compared to other ocular tissues and brain.Methods: Retina, RPE/Choroid, ciliary body, optic nerve and brain samples were collected from deceased human donors (N=7). GG were extracted and analysed by a comprehensive …