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Macronutrients
Published in Chuong Pham-Huy, Bruno Pham Huy, Food and Lifestyle in Health and Disease, 2022
Chuong Pham-Huy, Bruno Pham Huy
Glycosphingolipids are lipids containing at least one monosaccharide residue linked to ceramide moiety. They are found in brain and nervous tissue. Ceramides are amides of fatty acids which are long-chain saturated or monounsaturated fatty acids (138).
Hereditary and Metabolic Diseases of the Central Nervous System in Adults
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Fabry's disease is an X-linked lysosomal enzyme disorder of α-galactosidase, resulting in accumulation of glycosphingolipids with α-galactosyl moieties. The inability to process these glycosphingolipids results in their buildup in multiple organs. Most of the early, prominent symptoms are the result of accumulation in the blood vessels and the peripheral and central autonomic nervous system, although all tissues are affected. As the disease progresses, accumulation in kidneys and heart will lead to organ failure. Progressive arterial accumulations result in infarction. Fabry's disease usually affects males (1:60,000), but many heterozygous females have disease symptoms including strokes. Furthermore, some heterozygous females have symptoms as severe as the classic male phenotype due to nonrandom X-inactivation, where the unaffected X-chromosome is inactivated in more than 50% of cells, leading to more pronounced deficiency of α-galactosidase. Onset is late childhood (usual) into middle age.
Gaucher disease
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
The consequence of defective activity of acid β-glucosidase is the accumulation of glycosylceramide (Figure 90.1). In ceramide, there is a long-chain fatty acid amide linkage at the carbon 2 of sphingosine. Glycosphingolipids with longer oligosaccharide moieties are successively degraded to glycosylceramide. The amounts of this compound stored in Gaucher disease are enormous. Deacylated glycosylsphingosine has also been found in tissues of patients [85]. It is thought that the accumulated compounds are toxic to certain tissues.
Immunomodulatory regulation by heat-labile enterotoxins and potential therapeutic applications
Published in Expert Review of Vaccines, 2021
Mary-Peyton A. Knapp, Taylor A. Johnson, Madison K. Ritter, Robert O. Rainer, Steven E. Fiester, Jennifer T. Grier, Terry D. Connell, Sergio Arce
Gangliosides are a family of structurally diverse glycosphingolipids characterized by a ceramide core with linked oligosaccharides, which are decorated with one or more sialic acids. Gangliosides are anchored in the plasma membrane of cells and serve as signaling molecules [17,20,21]. These molecules are found ubiquitously on cells across body tissues but vary in expression in different cell types both qualitatively and quantitatively [22–24]. It has been demonstrated that the function of gangliosides in cell membranes influence cell differentiation [18,20,25,26], inhibit cytotoxic effects [18], trigger signal transduction pathways [24], and stimulate cell proliferation [27]. Studies are ongoing to reveal the roles of gangliosides in a number of pathological conditions [28,29] and their potential for use in developing immunotherapies [30].
Emerging pharmacological strategies for the management of chemotherapy-induced peripheral neurotoxicity (CIPN), based on novel CIPN mechanisms
Published in Expert Review of Neurotherapeutics, 2020
Andreas A. Argyriou, Jordi Bruna, Susanna B. Park, Guido Cavaletti
Gangliosides are glycosphingolipids, composed of a ceramide base with an oligosaccharide chain to which one or more sialic acids are bound. These are ubiquitous in outer layer of cell plasma membrane components, and highly abundant in the nervous system. Research into ganglioside function originates decades earlier, showing that gangliosides, especially the Ganglioside-monosialic acid GM1, are involved in a wide variety of neurotrophic and neuroprotective processes [46]. The best characterized GM1 mechanism of action suggests that exogenously supplied GM1 inserts in the membrane and modulates the neurotrophin tyrosine kinase receptors, Trk A and B, promoting cellular survival [47]. However, it should not be disregarded that some cancer cells, including colon adenocarcinoma, also express Trk receptors.
Double-edged role of natural killer cells during RSV infection
Published in International Reviews of Immunology, 2020
Rauf Bhat, Mohamed A. Farrag, Fahad N. Almajhdi
Various studies have highlighted the role of glycosphingolipids, particularly GM1 and Asialo-GM1, in RSV biology [51]. ASGM1 is up-regulated in the T-cells during viral infection. The RSV-specific and the IFN-γ-expressing CD8+ T cells have high GM1 expression in vivo. However, a decreased T-cell GM1 expression is marked by reduced viral clearance and lower disease severity [51, 52]. It was shown in mice that an RSV infection up-regulated the ASGM1 expression in the NK cells, but made no impact on the high GM1 expression. Anti-ASGM1 antibody treatment of the RSV-infected mice depleting the GM1/ASGM1-expressing NK and the T cells resulted in the reduced lung IFN-γ levels, delayed viral clearance, and diminished disease severity [52]. These data suggest that high expression of ASGM1-GM1 on NK and CD8 T cells may play a dominant role in the RSV-induced T-cell immunopathology [52]. However, the use of anti-sialo GM1 antibodies in BALB/c mice which are typically used for studying RSV pathogenesis pose a problem. The expression of ASGM1 is not strictly confined to NK cells but is also detected on basophils, a subpopulation of NKT, CD8+ T, γδ T cells, some activated CD4+ T cells, macrophages, and eosinophils. More specific anti-NK1.1 mAb as an NK cell depletion agent is suitable for use in only certain mouse strains such as C57BL/6 but not BALB/c mice [53].