But without gangliosides the txansparency of supernatant of injuried control erythrocytes is only 13.4% of the nounal value.The injuried erythrocytes shriek with spines on their surface. 而损伤对照组上清透光度仅为正常值的13.4%,红细胞胞体缩小,成为棘形红细胞。
This enzyme normally breaks down a lipid called GM2 ganglioside.
HEX-A正常情况下能降解一种叫做GM2神经节苷脂的脂质。
Osmosis-遗传
All right, as a quick recap, Tay-Sachs disease is a lysosomal storage disease that results in a buildup of GM2 ganglioside in neurons of the central nervous system.
A ganglioside is a molecule composed of a glycosphingolipid (ceramide and oligosaccharide) with one or more sialic acids (e.g. n-acetylneuraminic acid, NANA) linked on the sugar chain. NeuNAc, an acetylated derivative of the carbohydrate sialic acid, makes the head groups of gangliosides anionic at pH 7, which distinguishes them from globosides.