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The fundamental structure of a cerebroside is ceramide. Monoglycosyl and oligoglycosylceramides having a mono or polysaccharide bonded glycosidically to the terminal OH group of ceramide are defined as cerebrosides. Sphingosine is the main long-chain base present in ceramide. Galactosylceramide is the principal glycosphingolipid in brain tissue.
Ceramides are a family of waxy lipid molecules. A ceramide is composed of sphingosine and a fatty acid joined by an amide bond. Ceramides are found in high concentrations within the cell membrane of eukaryotic cells, since they are component lipids that make up sphingomyelin, one of the major lipids in the lipid bilayer. [1]
It is a component of the cell plasma membrane that modulates cell signal transduction events, and appears to concentrate in lipid rafts. [2] [3] Recently, gangliosides have been found to be highly important molecules in immunology. Natural and semisynthetic gangliosides are considered possible therapeutics for neurodegenerative disorders. [4]
The chemically synthesized compound N-(30hydroxy-1-hydroxymethyl-3-phenylpropyl)dodecamide (HPA-12) has been found to be an inhibitor of CERT-mediated ceramide trafficking. [21] More specifically, this drug inhibits the ATP-dependent transport of ceramide from ER to Golgi (and therefore the conversion of ceramide to sphingomyelin), but it does ...
β-Glucocerebrosidase (also called acid β-glucosidase, D-glucosyl-N-acylsphingosine glucohydrolase, or GCase) is an enzyme with glucosylceramidase activity (EC 3.2.1.45) that cleaves by hydrolysis the β-glycosidic linkage of the chemical glucocerebroside, an intermediate in glycolipid metabolism that is abundant in cell membranes (particularly skin cells). [5]
The latter can be distinguished again by means of the charge carrier. While in gangliosides sialic acids are found, sulfatides have a sulfate group. The structural similarity of most glycolipids is the so-called lactosylceramide, that is, a lactose disaccharide that is glycosidically bound to a ceramide.
The long-chain bases, sometimes simply known as sphingoid bases, are the first non-transient products of de novo sphingolipid synthesis in both yeast and mammals. These compounds, specifically known as phytosphingosine and dihydrosphingosine (also known as sphinganine, [4] although this term is less common), are mainly C 18 compounds, with somewhat lower levels of C 20 bases. [5]
Ceramide glucosyltransferase (or glucosylceramide synthase) is a glucosyltransferase enzyme involved in the production of glucocerebrosides (also called glucosylceramides). ). It is responsible for the first step in synthesis of all glycosphingolipids—critical components of cell membranes—from sphingolipids, attaching a glucose molecule (glycosylation) to produce a glucocerebroside product