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Activation of the immune response in Fabry disease

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Globotriaosylceramide and globotriaosylsphingosine influence the levels of soluble factors and signaling receptors and the inflammatory-cell profile in Fabry disease. Different immune responses may be triggered in Fabry disease patients of different genders or distinct disease manifestations. Fabry disease treatment, specifically enzyme replacement treatment, also influences the immune response.

Abbreviations: BNP, B-type natriuretic peptide; CCR2, monocyte chemoattractant protein-1 receptor; CD, cluster of differentiation; DCs, dendritic cells; DD, diastolic dysfunction; DN iNKT, double-negative invariant natural killer T cells; ERT, enzyme replacement treatment; FGF2, fibroblast growth factor 2; Gb-3, globotriaosylceramide; GM-CSF, granulocyte-macrophage-colony-stimulating factor; IL, interleukin; INF-γ, interferon-gamma; iNKT, invariant natural killer T cells; LGE, late gadolinium enhancement; LVH: left-ventricular hypertrophy; Lyso-Gb3, globotriaosylsphingosine; MCP-1, Monocyte Chemoattractant Protein-1; MHC, major histocompatibility complex; MMP: metalloproteinase; MR-proANP, midregional pro-atrial natriuretic peptide; No ≠: no differences; TGF, transforming growth factor; TLR, Toll-like receptor; TNF, tumor necrosis factor; TNFR, tumor necrosis factor receptor.

(Source: Coelho-Ribeiro B, Silva HG, Sampaio-Marques B, et al. Inflammation and exosomes in Fabry disease pathogenesis. Cells 2024;13[8]:654. Adapted from: Carnicer-Cáceres C, Arranz-Amo JA, Cea-Arestin C, et al. Biomarkers in Fabry disease. Implications for clinical diagnosis and follow-up. J Clin Med 2021;10[8]:1664. Creative Commons Attribution 4.0 International [CC BY 4.0] license, creativecommons.org/licenses/by/4.0.)