Booth D A, Goodwin H, Cumings J N
J Lipid Res. 1966 May;7(3):337-40.
The molar ratios of N-acetyl neuraminic acid, hexose, hexosamine, and sphingosine have been determined for the abnormal ganglioside in Tay-Sachs disease that was previously detected as a fast-moving band in thin-layer chromatography, and in two abnormal fast-moving bands of gangliosides from the cortex and white matter of the brain in cases of gargoylism and Niemann-Pick's disease. The fastest-moving ganglioside band in these two conditions contains neither hexosamine nor glucose.
已测定了泰-萨克斯病中异常神经节苷脂的N-乙酰神经氨酸、己糖、己糖胺和鞘氨醇的摩尔比,该异常神经节苷脂先前在薄层色谱中被检测为快速移动条带;还测定了在脂肪代谢障碍和尼曼-皮克病病例中,大脑皮质和白质中神经节苷脂的两条异常快速移动条带的上述摩尔比。在这两种情况下,移动速度最快的神经节苷脂条带既不含己糖胺也不含葡萄糖。