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Lysosomal Storage Disorders and Enzyme Replacement Therapy
Published in Peter Grunwald, Pharmaceutical Biocatalysis, 2020
Niemann-Pick disease, Fabry disease, Krabbe disease, Gaucher disease, Tay-Sachs disease and metachromatic leukodystrophy (MLD), and Faber and Sandhoff disease are members of this group of diseases. As the name suggests, they are all related to the sphingolipid metabolism. Sphingolipids (or glycosylceramides) play important roles in cell recognition and signaling pathways based on their ability to interact with cell surface proteins (lectins, receptor proteins, etc.), as summarized in an excellent review by Wennekes et al. (2009). Recent studies have elucidated the enormous complexity they contribute to the lipidome (the totality of lipids in cells) and its many functions in health and disease (Hannun et al., 2018).
Nanonutraceutical Delivery Systems
Published in Pradipta Ranjan Rauta, Yugal Kishore Mohanta, Debasis Nayak, Nanotechnology in Biology and Medicine, 2019
Bioactive lipids play essential roles as signaling and regulatory molecules. In particular, sphingolipids, which form a major component of cell membranes, play a vital role in regulating cell transport across the membrane, cellular growth, senescence, and adhesion (Hannun and Obeid, 2008). Medium-chain triglycerides comprising six to eight carbons help in weight reduction, alleviate a wide range of cardiovascular ailments, and serve as an ideal candidate in preventing Type 2 diabetes. Oleic acid is advocated in the reduction of neurodegenerative diseases such as dementia and Alzheimer’s disease and also in the management of colorectal cancer. Polyunsaturated fatty acids such as linolenic and linoleic acids have broad health-promoting beneficial effects such as protection against chronic diseases such as cardiovascular disorders, diabetes, cancer, atherosclerosis, platelet aggregation, and a variety of disorders caused by inflammation (Aluko, 2012).
Molecular toxicology and carcinogenesis of fumonisins: a review
Published in Journal of Environmental Science and Health, Part C, 2020
Ruth Nabwire Wangia-Dixon, Kizito Nishimwe
Mice deficient of ceramide synthase which is inhibited by fumonisin B1, have been shown to spontaneously develop liver tumors which supports the science that disruption of sphingolipid profile can potentiate carcinogenesis via disruption of cell cycle regulation.92,111 The current understanding of fumonisin B1 induced mechanism of carcinogenesis involves sphingolipid signaling pathways. This indicates that the balance between intracellular concentrations of sphingolipid effectors that protect cells from apoptosis and the effectors that induce apoptosis determines cellular response.11,13 The balance between the rates of apoptosis and proliferation is a critical determinant in hepato- and nephrotoxicity and tumorigenesis in animal models.92 Therefore, contamination of food supplies for human consumption should be as low as reasonably possible to mitigate the increased risk of tumorigenesis associated with exposure to fumonisins.
Ceramide pathway: A novel approach to cancer chemotherapy
Published in Egyptian Journal of Basic and Applied Sciences, 2018
Mahdi Mashhadi Akbar Boojar, Masoud Mashhadi Akbar Boojar, Sepide Golmohammad
Sphingolipids are a class of lipids with sphingosine as a complex amino alcohol core containing 18 carbons, which together with glycerolipids and sterols, form the major part of the cell membrane structure. Ceramide as ubiquitous sphingolipids is composed of a sphingosine, which is amide-bonded to a fatty acyl chain with different numbers of carbons, ranging from 14 to 26 [16]. Ceramides are one of the major components of the cell membrane and play a key role in protecting the cell from environmental stress [11]. The addition of phosphoethanolamine, monosaccharide or oligosaccharide together with sialic acid to ceramide leads to the formation of sphingomyelin, cerebrosides, and ganglioside, which, apart from the structural importance, they play a role as biological markers and the binding agent of extracellular ligands to the receptor [3,17] .
An expedient, chemoselective N-chloroacetylation of aminoalcohols under metal-free bio-compatible conditions
Published in Green Chemistry Letters and Reviews, 2018
Sphingolipids (8, 9) belong to an important class of lipids that are present in all membranes, especially in the myelin sheath. Structurally, these compounds consist of two major units, sphingoid bases (10) (sphingosine, an aliphatic amino alcohols unit) and a fatty acid. Sphingolipids play vital roles in many cellular functions (9, 11, 12) including, signal transmission and cell recognition. A sphingolipid with an R group consisting of a hydrogen atom only is a ceramide. Ceramides are of commercial and therapeutic utility (13). Currently, ceramides, are primarily obtained via extraction and isolation from animal sources. The presence of other similar compounds makes isolation of ceramides very difficult. It would thus be desirable to find an alternative, cost-effective, high-yield method for obtaining these valuable products. Thus, chemical synthesis methods may provide a suitable alternative (14–18).