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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]
The structure also revealed significant characteristics of the protein important to its function such as the reentrant membrane helix (blue/light blue) that dips into the first leaflet of the membrane as well as the highly conserved Asp-Glu catalytic dyad within the active site (green loop). [8]
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.
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 ...
This can be done in terms of the chemical elements present, or by molecular structure e.g., water, protein, fats (or lipids), hydroxyapatite (in bones), carbohydrates (such as glycogen and glucose) and DNA. In terms of tissue type, the body may be analyzed into water, fat, connective tissue, muscle, bone, etc.
Antibodies to ganglioside are found to be elevated in coeliac disease. [16] Recent studies show that gliadin can cross-link to gangliosides in a transglutaminase independent manner, indicating that gliadin specific T-cell could present these antigens to the immune system. [17]
Plasmalogens are found in numerous human tissues, with particular enrichment in the nervous, immune, and cardiovascular systems. [1] [2] [3] In human heart tissue, nearly 30–40% of choline glycerophospholipids are plasmalogens. Even more striking is the fact that 32% of the glycerophospholipids in the adult human heart and 20% in brain and up ...