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FIGURE 1

FIGURE 1. A schematic for a model of inflammatory propagation of Gaucher. From: Immunological Cells and Functions in Gaucher Disease.

MΦ activation due to excess of glucosylceramide (GC) could trigger the release of C-C chemokines, e.g., monocyte chemoat-tractant protein-1(MCP1) or CC chemokine ligand-2 (CCL2),which cause the recruitment of blood MOs into the different visceral organs. These cells then mature into MΦs and DCs subsets. Because of the GCase defects in these cells, excess of GC accumulates and in turn activates the release of interferon-γ (IFN-γ), interleukin-4 (IL-4), IL-6, and transforming growth factor-β (TGF-β). The cytokines IFN-γ and Il-4 cause the development of T helper-1 (Th1) and Th2 cell-mediated responses, whereas IL-6 facilitates the development of follicular T cells (Tfh). These responses lead to the formation and activation of the germinal center that triggers B-cell differentiation and immunoglobulin (IgG, IgA, and IgM) production, and hypergammaglobulinemia. IL-6 together with TGF-β impact Th17 cell development, which induces the production of IL-17 and subsequently the production of CXCL8/IL8 to recruit blood PMNs into Gaucher disease visceral organs. In addition to CXCL8/IL8, GC-engorged MΦs also secrete KC/CXCL1, IL-1β, IFN-γ, and TNF-α, which are critical for the recruitment of PMNs and release of their activation products (e.g., TNF-α, IL-6, IL-1α, IL-1β, and IL-1Ra) into the visceral organs. Also, TNF-α together with IFN-γ and IL-1β induce iNOS followed by the production of NO to trigger immunological inflammation in Gaucher disease.

Manoj Kumar Pandey, et al. Crit Rev Oncog. ;18(3):197-220.

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