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The effect of Globotriaosylceramide on invariant Natural Killer T cell activation .

Investigador: Maria de Fátima Macedo
Instituição: UniLiPE, IBMC – Porto



Fabry disease is a lysosomal storage disease (LSD) characterized by the accumulation of globotriaosylceramide (Gb3) in the late endosomes and lysosomes, due to deficiency of the enzyme α-Galactosidase A.
Invariant Natural Killer T (iNKT) cells are lipid specific T cells that play important roles in infection, cancer and auto-immunity.

We have previously described phenotypical and functional alterations in iNKT cells of both Fabry disease patients and mice (Macedo et al. 2012; Pereira et al. 2013), which were in agreement with a pro-inflammatory state suggested in Fabry disease (De Francesco et al. 2013).

As glycosphingolipids are arising as important regulators of the immune response, we hypothesized that Gb3 might exert a direct effect in iNKT cell regulation. In fact, sulfatide was shown to inhibit iNKT cell activation, through competitive binding to CD1d (Kanamori et al. 2012). Therefore, the aim of this project is to investigate whether a similar role can be attributed to Gb3 on CD1d-mediated lipid antigen presentation and consequently on iNKT cell function.

This work will contribute to clarify the role of Gb3 in iNKT cell function and may contribute to the definition of a new mechanism explaining the iNKT cell abnormalities found in other LSDs.



Faculdade de Farmácia da U.L.
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Contacto: Fernanda Asper
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