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More than 60 gangliosides are known, which differ from each other mainly in the position and number of NANA residues. 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 ...
The numbering is based on its relative mobility in electrophoresis among other monosialic gangliosides. [1] Its structure can be condensed to NANA - Gal - Glc - ceramide . GM3 is the most common membrane-bound glycosphingolipid in tissues, composed of three monosaccharide groups attached to a ceramide backbone. [ 2 ]
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.
This enzyme catalyzes the biodegradation of fatty acid derivatives known as gangliosides. [1] The diseases are better known by their individual names: Tay–Sachs disease, AB variant, and Sandhoff disease. Beta-hexosaminidase is a vital hydrolytic enzyme, found in the lysosomes, that breaks down lipids. When beta-hexosaminidase is no longer ...
The GM1 gangliosidoses, usually shortened to GM1, are gangliosidoses caused by mutation in the GLB1 gene resulting in a deficiency of beta-galactosidase.The deficiency causes abnormal storage of acidic lipid materials in cells of the central and peripheral nervous systems, but particularly in the nerve cells, resulting in progressive neurodegeneration.
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 structure of sphingomyelin was first reported in 1927 as N-acyl-sphingosine-1-phosphorylcholine. [3] Sphingomyelin content in mammals ranges from 2 to 15% in most tissues, with higher concentrations found in nerve tissues, red blood cells, and the ocular lenses. Sphingomyelin has significant structural and functional roles in the cell.
An osteocyte, an oblate shaped type of bone cell with dendritic processes, is the most commonly found cell in mature bone. It can live as long as the organism itself. [1] The adult human body has about 42 billion of them. [2]