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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]
The structural similarity of most glycolipids is the so-called lactosylceramide, that is, a lactose disaccharide that is glycosidically bound to a ceramide. Larger structures are subdivided into different groups by the sequence and configuration of the sugars, the four most common being globo-, lacto-, neoLacto- and gangliose.
Glycolipid. Glycolipids are lipids with a carbohydrate attached by a glycosidic (covalent) bond. [1] Their role is to maintain the stability of the cell membrane and to facilitate cellular recognition, which is crucial to the immune response and in the connections that allow cells to connect to one another to form tissues. [2]
The heads of glycolipids (glyco- stands for sugar) contain a sphingosine with one or several sugar units attached to it. The hydrophobic chains belong either to: two fatty acids (FA) – in the case of the phosphoglycerides, or; one FA and the hydrocarbon tail of sphingosine – in the case of sphingomyelin and the glycolipids.
The primary saponifiable lipids are free fatty acids, neutral glycerolipids, glycerophospholipids, sphingolipids, and glycolipids. [ 1 ] By comparison, the non-saponifiable class of lipids is made up of terpenes, including fat-soluble A and E vitamins, and certain steroids, such as cholesterol.
Constitutive degradation of sphingolipids and glycosphingolipids takes place in the acidic subcellular compartments, the late endosomes and the lysosomes, with the end goal of producing sphingosine. In the case of glycosphingolipids, exohydrolases acting at acidic pH optima cause the stepwise release of monosaccharide units from the end of the ...
1 in 100,000 [3] Limited Highly variable, infantile neurovisceral Niemann Pick disease (Type A ASMD) is usually fatal before 3 years of age. Estimasted mortality before adulthood for the Chronic visceral form (type B) is around 15-25%. Many live well into adulthood and may reach a normal lifespan. Diagnosis have been made in the 7th decade of life.
[1] [2] In humans, SPH represents ~85% of all sphingolipids, and typically makes up 10–20 mol % of plasma membrane lipids. Sphingomyelin was first isolated by German chemist Johann L.W. Thudicum in the 1880s. [3] The structure of sphingomyelin was first reported in 1927 as N-acyl-sphingosine-1-phosphorylcholine. [3]