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10.4049/jimmunol.1201483 http://scihub22266oqcxt.onion/10.4049/jimmunol.1201483 22875802!3442250!22875802     free     free     free Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 267.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 267.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 267.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 267.2 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       J+Immunol 2012 ; 189 (6): 3007-17 Nephropedia Template TP {{tp|p=22875802|t=2012. Globosides but not isoglobosides can impact the development of invariant NKT cells and their interaction with dendritic cells |pdf=|usr=}}{{22875802}} gab.com Text Globosides but not isoglobosides can impact the development of invariant NKT cells and their interaction with dendritic cells JO: J Immunol http://www.kidney.de/pget.php?&tw=1&pmid=22875802 #FOAvip Recognition of endogenous lipid Ag(s) on CD1d is required for the development of invariant NKT (iNKT) cells. Isoglobotrihexosylceramide (iGb3) has been implicated as this endogenous selecting ligand and recently suggested to control overstimulation and deletion of iNKT cells in alpha-galactosidase A-deficient (alphaGalA(-/-)) mice (human Fabry disease), which accumulate isoglobosides and globosides. However, the presence and function of iGb3 in murine thymus remained controversial. In this study, we generate a globotrihexosylceramide (Gb3)-synthase-deficient (Gb3S(-/-)) mouse and show that in thymi of alphaGalA(-/-)/Gb3S(-/-) double-knockout mice, which store isoglobosides but no globosides, minute amounts of iGb3 can be detected by HPLC. Furthermore, we demonstrate that iGb3 deficiency does not only fail to impact selection of iNKT cells, in terms of frequency and absolute numbers, but also does not alter the distribution of the TCR CDR 3 of iNKT cells. Analyzing multiple gene-targeted mouse strains, we demonstrate that globoside, rather than iGb3, storage is the major cause for reduced iNKT cell frequencies and defective Ag presentation in alphaGalA(-/-) mice. Finally, we show that correction of globoside storage in alphaGalA(-/-) mice by crossing them with Gb3S(-/-) normalizes iNKT cell frequencies and dendritic cell (DC) function. We conclude that, although detectable in murine thymus in alphaGalA(-/-)/Gb3S(-/-) mice, iGb3 does not influence either the development of iNKT cells or their interaction with peripheral DCs. Moreover, in alphaGalA(-/-) mice, it is the Gb3 storage that is responsible for the decreased iNKT cell numbers and impeded Ag presentation on DCs. Twit Text FOAVip Globosides but not isoglobosides can impact the development of invariant NKT cells and their interaction with dendritic cells ????J Immunol http://www.kidney.de/pget.php?&tw=1&pmid=22875802 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=22875802 #FOAvip English Wikipedia <ref>{{Cite pmid|22875802}}</ref> Globosides but not isoglobosides can impact the development of invariant NKT cells and their interaction with dendritic cells #MMPMID22875802 Porubsky S; Speak AO; Salio M; Jennemann R; Bonrouhi M; Zafarulla R; Singh Y; Dyson J; Luckow B; Lehuen A; Malle E; Muthing J; Platt FM; Cerundolo V; Grone HJ J Immunol 2012[Sep]; 189 (6): 3007-17 PMID22875802show ga Recognition of endogenous lipid Ag(s) on CD1d is required for the development of invariant NKT (iNKT) cells. Isoglobotrihexosylceramide (iGb3) has been implicated as this endogenous selecting ligand and recently suggested to control overstimulation and deletion of iNKT cells in alpha-galactosidase A-deficient (alphaGalA(-/-)) mice (human Fabry disease), which accumulate isoglobosides and globosides. However, the presence and function of iGb3 in murine thymus remained controversial. In this study, we generate a globotrihexosylceramide (Gb3)-synthase-deficient (Gb3S(-/-)) mouse and show that in thymi of alphaGalA(-/-)/Gb3S(-/-) double-knockout mice, which store isoglobosides but no globosides, minute amounts of iGb3 can be detected by HPLC. Furthermore, we demonstrate that iGb3 deficiency does not only fail to impact selection of iNKT cells, in terms of frequency and absolute numbers, but also does not alter the distribution of the TCR CDR 3 of iNKT cells. Analyzing multiple gene-targeted mouse strains, we demonstrate that globoside, rather than iGb3, storage is the major cause for reduced iNKT cell frequencies and defective Ag presentation in alphaGalA(-/-) mice. Finally, we show that correction of globoside storage in alphaGalA(-/-) mice by crossing them with Gb3S(-/-) normalizes iNKT cell frequencies and dendritic cell (DC) function. We conclude that, although detectable in murine thymus in alphaGalA(-/-)/Gb3S(-/-) mice, iGb3 does not influence either the development of iNKT cells or their interaction with peripheral DCs. Moreover, in alphaGalA(-/-) mice, it is the Gb3 storage that is responsible for the decreased iNKT cell numbers and impeded Ag presentation on DCs. |*Trihexosylceramides/deficiency/physiology[MESH] |Animals[MESH] |Carbohydrate Sequence[MESH] |Cell Differentiation/*immunology[MESH] |Dendritic Cells/enzymology/*immunology/metabolism[MESH] |Globosides/deficiency/*physiology[MESH] |Liver/cytology/enzymology/metabolism[MESH] |Mice[MESH] |Mice, 129 Strain[MESH] |Mice, Inbred C57BL[MESH] |Mice, Knockout[MESH] |Mice, Transgenic[MESH] |Molecular Sequence Data[MESH] |Natural Killer T-Cells/enzymology/*immunology/metabolism[MESH] |Spleen/cytology/enzymology/metabolism[MESH] |Thymus Gland/cytology/enzymology/metabolism[MESH]Globosides but not isoglobosides can impact the development of invaria(epmc).pdf DeepDyve Pubget Overpricing