Ganglioside GM2 asialo (asialo-GM2) is a glycosphingolipid containing three monosaccharide residues and one fatty acid of variable chain length, but lacks the sialic acid residue present on ganglioside M2. Asialo-GM2 is found at low or undetectable levels in normal human brains, but it accumulates in the brains of patients with Tay-Sachs disease and Sandhoff disease, which are expressed as lysosomal β- A neurodegenerative disorder characterized by hexosaminidase A and B deficiency. It also binds to various bacteria, including Pseudomonas isolated from cystic fibrosis patients. The Asialo-GM2 mixture contains ganglioside GM2 asialo molecular species with fatty acyl chains of variable length.
CAS
35960-33-9
中文名称
GA2神经节苷脂
分子量
1093.43
运输条件
Room temperature in continental US; may vary elsewhere.
保存条件
Please store the product under the recommended conditions in the Certificate of Analysis.
产品信息
Intro
神经节苷脂 GM2 asialo (asialo-GM2) 是一种糖鞘脂,含有三个单糖残基和一个链长可变的脂肪酸,但缺少神经节苷脂 M2 上存在的唾液酸残基。 Asialo-GM2 在正常人脑中的水平很低,甚至检测不到,但它会在泰-萨克斯病和桑德霍夫病患者的大脑中积累,这两种疾病是分别以溶酶体 β-己糖胺酶 A 和 B 缺乏为特征的神经退行性疾病。它还与各种细菌结合,包括从囊性纤维化患者分离出的假单胞菌。 Asialo-GM2 混合物包含神经节苷脂 GM2 asialo 分子种类,具有可变长度的脂肪酰链。