Globosides/红细胞糖苷酯/11034-93-8
Specifications
Catalog #:1068
Scientific Name:Globosides
Common Name:Gb4; Globotetrahexosylceramide
Empirical Formula:C68H126N2O23 (tetracosanoyl)
Typical fatty acid contentCAS#:11034-93-8
SDS:View Safety Data Sheet
Data Sheet:View Data Sheet
Formula Weight:1340 (tetracosanoyl)
Unit:5 mg
Solvent:none
Source:natural
Purity:98+%
Analytical Methods:TLC; identity confirmed by MS
Natural Source:porcine RBC
Solubility:chloroform/methanol, 2:1; DMSO, hot methanol
Physical Appearance:solid
Storage:-20℃
Dry Ice:No
Hazardous:No
Description
Application Notes:
球糖苷是红细胞膜中最丰富的中性糖脂,聚集在细胞壁脂筏中。它们参与细胞通讯,增加细胞粘附和作为受体的功能。它是血型p抗原的基本结构。该抗原的缺陷导致抗球蛋白抗体和P血型表型。球形糖苷是多种毒素的受体,包括p -缨状大肠杆菌,人细小病毒B191和白菜蝴蝶的pierisin-1毒素在泰-萨克斯病和山德霍夫病中,由于β -己糖苷酶的缺乏,球蛋白可累积。
Globoside is the most abundant neutral glycolipid in the erythrocyte membrane accumulating in lipid rafts of the cell wall. They are involved in cell communication, increase cell adhesion and function as receptors. It is an essential structure of the blood group P-antigen. A deficiency in this antigen leads to anti-globoside antibodies and the P blood group phenotypes. Globoside is a receptor for multiple toxins including P-fimbriated Escherichia coli, human parvovirus B191 and pierisin-1 toxin from the cabbage butterfly.2 In Tay-Sachs and Sandhoff disease globoside can accumulate due to the deficiency of the beta-hexosaminidase.3
References:
1. Boel Lanne et al, The Journal of Biological Chemistry, Vol. 270 pp. 9017, 1995
2. Yuko Matsushima-Hibiya, The Journal of Biological Chemistry, Vol. 278 pp. 9972, 2003
3. R. A. Gravel, M. M. Kaback, R. Proia, K. Sandhoff, K. Suzuki, and K. Suzuki. in The Metabolic and Molecular Bases of Inherited Disease (C. R. Scriver, W. S. Sly, B. Childs, A. L. Beaudet, D. Valle, K. W. Kinzler, and B. Vogelstein, eds) pp. 3827–3876, McGraw-Hill Inc., New York, 2001