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Krabbe disease/galactosylceramide lipidosis/globoid cell leukodystrophy
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
The structure of galactosylceramide is shown in Figure 93.1. Cerebrosides are monohexosyl ceramides in which the sugar is glycosidically linked to the C-1 of ceramide. Galactosylceramide is the characteristic cerebroside of myelin and of the central nervous system. The compound is normally degraded to ceramide and galactose by the lysosomal enzyme galactosylceramide β-galactosidase [6]. In patients, the level of activity has been documented to be 5–10 percent normal in brain, liver, spleen, and kidney [6, 7]. The assay is conveniently and reliably performed on leukocytes or cultured fibroblasts [59]. Enzymatic diagnosis with the natural substrate is demanding and should be carried out in an experienced laboratory [57, 60].
Recent Insights on the Role of Natural Medicines in Immunostimulation
Published in Dilip Ghosh, Pulok K. Mukherjee, Natural Medicines, 2019
Isabella Muscari, Sabrina Adorisio, Trinh Thi Thuy, Tran Van Sung, Domenico V. Delfino
Agelasphins. These constitute a class of natural glycolipids derived from the Okinawan marine sponge Agelas mauritianus, which has potent anti-tumour activity as a consequence of robust immunostimulation. Even more potent immunostimulants in the form of a simple α-galactosylceramide (α-GalCer) have been developed from these glycolipids. This compound was further modified to increase the immunostimulatory effect, resulting in a potent CD1d ligand that stimulates mouse invariant natural killer T (iNKT) cells to selectively enhance Th1 cytokine production (Hossain et al. 2016).
Invariant natural killer T cells minimally influence gut microbiota composition in mice
Published in Gut Microbes, 2022
Qiaochu Lin, Meggie Kuypers, Zhewei Liu, Julia K Copeland, Donny Chan, Susan J Robertson, Jean Kontogiannis, David S Guttman, E. Kate Banks, Dana J Philpott, Thierry Mallevaey
Mice were used between 6–8 weeks of age, unless otherwise specified. C57BL/6 (B6) mice were purchased from Jackson Laboratories. CD1d−/− mice were generated and generously provided by Dr. Chyung-Ru Wang (Northwestern University, USA).87 Vα14 Tg mice were a generous gift from Dr. Albert Bendelac (University of Chicago).49 All strains were bred at the Division of Comparative Medicine, University of Toronto animal facility under barrier conditions in separate designated areas, with either specific-pathogen free (SPF) or enhanced (e) SPF status. The mice used in this study were the product of in-house heterozygous breeding pairs or triads, unless specified. All animal procedures were approved by the Faculty of Medicine and Pharmacy Animal Care Committee at the University of Toronto (Animal use protocols 20010135, 20010715, 20011113, 20011656). α-galactosylceramide (KRN7000, αGC) was purchased from Diagnocine. Antibodies used were purchased from ThermoFisher Scientific, Biolegend or BD Biosciences. OCH9, as well as PBS57-loaded and unloaded biotinylated CD1d monomers were obtained from the NIH Tetramer Core Facility. CD1d monomers were tetramerized by addition of fluorochrome-conjugated streptavidin.
A review on neuropharmacological role of erucic acid: an omega-9 fatty acid from edible oils
Published in Nutritional Neuroscience, 2022
J. B. Senthil Kumar, Bhawna Sharma
Galactosylceramides (Figure 1(G)) and its sulphated version called sulfatide with long-chain fatty acid moieties, in particular 24:0 and 24:1 fatty acids, are considered to be the most typical myelin lipids [45]. The incorporation of these type of saturated and long-chain fatty acid are believed to influence the membrane thickness and the packing density of lipids within myelin. Mice that lack the enzyme UDP-galactose ceramide galactosyltransferase (CGT) (transfers galactose to ceramide in the biosynthesis of galactocerebroside) do not synthesise galactosylceramide and sulfatide and develop severe neurological deficits a few weeks after birth [41]. Similarly, a decrease in sulfogalactosyl ceramide (Figure 1(G)) has been reported in both grey matter and white matter of post mortem brains from AD subjects with mild dementia.
Synthesis and structure-activity relationships of cerebroside analogues as substrates of cerebroside sulphotransferase and discovery of a competitive inhibitor
Published in Journal of Enzyme Inhibition and Medicinal Chemistry, 2020
Wenjin Li, Joren Guillaume, Younis Baqi, Isabell Wachsmann, Volkmar Gieselmann, Serge Van Calenbergh, Christa E. Müller
The synthesised compounds were studied using a previously developed capillary electrophoresis-based assay, in which the conversion of the co-substrate PAPS, acting as a sulphate donor, to adenosine-3′,5′-bisphosphate (PAP) was measured18. In addition to the natural substrate galactosylceramide (3), which contains a β-galactosyl residue and is present in nerve tissues as a constituent of myelin, the corresponding β-glucosyl derivative glucocerebroside (9) was investigated, which is mainly found in liver and spleen. As another naturally occurring sphingolipid psychosine (galactosyl-β-sphingosine, 8) was investigated, which is a cytotoxic derivative of galactosylceramide (3) that lacks its fatty acid residue. Moreover, 13 glycolipids, four of which (12, 17, 20 and 22) are new compounds, not previously described in literature, were investigated as artificial substrate analogues and/or competitive inhibitors of CST (Table 1). Enzyme kinetic parameters (Michaelis–Menten constant and maximal velocity) were determined for all (artificial) substrates for which significant conversion of >20% as compared to the natural substrate (set at 100%) was observed.