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Lysosomal Storage Disorders and Enzyme Replacement Therapy
Published in Peter Grunwald, Pharmaceutical Biocatalysis, 2020
Due to variations in the carbohydrate composition and structure, and different fatty acid residues (chain length, double bonds, OH-groups) up to now more than 500 specific GSLs have been identified. A ceramide scaffolding with only one sugar moiety is named a cerebroside, e.g., galacto(syl)cerebrosides found in neuronal cell membranes, whereas glucocerebrosides are present in all other tissues. GSLs are termed globosides if the head group (X) is made up by more than one sugar residues (e.g., Gal, GalNac, Glc). A characteristic feature of so-called gangliosides, having potential as therapeutic tools for the treatment of various chronic and acute neurodegenerative diseases (Mocchetti, 2005; Lopez and Báez, 2018), is that at least one of the terminal sugar units is the N-acetylneuraminic acid (Neu5NAc) or sialic acid, negatively charged at pH = 7. For gangliosides, the Svennerholm abbreviations are often used (Carstea, 1997). In GM2, the letter G is the abbreviation for gangliosides; the letters M, D, T, Q, … indicate whether a mono-, di-, tri-, quartosialic acid is present in the molecule. The added number informs about the individual type of carbohydrate chain attached to the ceramide and is derived from the migration order of the respective ganglioside under certain experimental conditions (Svennerholm, 1963). Though not very systematic, this nomenclature has the advantage of being short; e.g., the structure of the ganglioside GM2 is GalNAcβ4(Neu5Acα3)Galβ4Glc Cer (Grunwald, 2016a).
Glossary of scientific and technical terms in bioengineering and biological engineering
Published in Megh R. Goyal, Scientific and Technical Terms in Bioengineering and Biological Engineering, 2018
Tay-Sachs disease is a lethal hereditary disease. The progressive accumulation of a substance called ganglioside in the brain causes paralysis, mental deterioration and blindness. Death usually occurs before the age of four.
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] .