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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 secreted toxin attaches to the surface of the host mucosa cell by binding to GM1 gangliosides. GM1 consists of a sialic acid-containing oligosaccharide covalently attached to a ceramide lipid. The A1 subunit of this toxin will gain entry to intestinal epithelial cells with the assistance of the B subunit via the GM1 ganglioside receptor.
[5] [6] Gangliosides can be found on neuronal cells and are membrane proteins that aid in cell-cell recognition and communication. PglC belongs to a superfamily of enzymes known as monotopic phosphoglycsoyl transferases (monoPGT).
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 ...
Bone marrow transplant (high risk, potential failure, effectively provides enzyme replacement to the central nervous system from six months post-transplant, if done in the earliest stages; less effective enzyme replacement provision for the peripheral nervous system)
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.