determination-tolerable-fatty-acids-cholera-toxin-concentrations

Determination of Tolerable Fatty Acids and Cholera Toxin Concentrations Using Human Intestinal Epithelial Cells and BALB/c Mouse Macrophages

The positive role of fatty acids in the prevention and alleviation of non-human and  human diseases have been and continue to be extensively documented. ...

Kingsborough Community College

Within the group of lysosomal storage diseases, NPC1 [NPC (Niemann-Pick type C) 1] disease is a lipidosis characterized by excessive accumulation of free cholesterol as well as gangliosides, glycosphingolipids and fatty acids in the late E/L (endosomal/lysosomal) system (Chen et al., 2005) due to a defect in late endosome lipid egress. We have previously demonstrated that expression of the small GTPase Rab9 in NPC1 cells can rescue the lipid transport block phenotype (Walter et al., 2003), albeit by an undefined mechanism.

Endosomal lipid accumulation in NPC1 leads to inhibition of PKC, hypophosphorylation of vimentin and Rab9 entrapment.

Au sein du groupe des maladies lysosomales, NPC1 [NPC (Niemann-Pick de type C) 1] la maladie est une lipidose caract&eacute;ris&eacute;e par une accumulation excessive de cholest&eacute;rol libre, mais aussi des gangliosides, glycosphingolipides et acides gras dans la fin E/L (endosomal /) syst&egrave;me lysosomal (Chen et coll., 2005) due &agrave; un d&eacute;faut dans l&#39;&eacute;vacuation de lipides endosome tardif. Nous avons d&eacute;montr&eacute; pr&eacute;c&eacute;demment que l&#39;expression de la petite GTPase Rab9 dans les cellules NPC1 peut sauver le ph&eacute;notype de bloc de transport de lipides (Walter et al., 2003), mais par un m&eacute;canisme non d&eacute;fini.

Endosomale accumulation de lipides dans NPC1 conduit &agrave; une inhibition de la PKC, hypophosphorylation de vimentine et des Rab9.

All&#39;interno del gruppo delle malattie da accumulo lisosomiale, NPC1 [NPC (Niemann-Pick tipo C) 1] la malattia &egrave; una lipidosi caratterizzata da eccessivo accumulo di colesterolo libero, come pure i gangliosidi, Solfatati e acidi grassi nel tardo E/L (endosomal/lisosomiale) sistema (Chen et al., 2005) a causa di un difetto nel tardo endosome uscita di lipidi. Abbiamo precedentemente dimostrato che espressione del piccolo GTPase Rab9 nelle cellule NPC1 pu&ograve; salvare il fenotipo di blocco del trasporto di lipidi (Walter et al., 2003), seppure da un meccanismo definito.

Farshad Tamari

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