Kennaway N G, Woolf L I
J Lipid Res. 1968 Nov;9(6):755-65.
Column chromatography (on cellulose, silicic acid, and Florisil) and thin-layer chromatography were employed for the separation and purification of lipid fractions from normal and Gaucher spleens. A new hydrolysis procedure, followed by paper chromatography, was used for identification of sugar moieties. A nonhydrolytic combined colorimetric procedure, with anthrone and orcinol, was used for the estimation of glucose and galactose separately in glycolipids. The limitations of this method were examined. Spleens from two control subjects and three patients with Gaucher's disease have been examined in detail. In all Gaucher spleens, the predominant feature was the massive acccumulation of glucocerebroside; neutral ceramide oligohexoside levels were probably within the normal range, as were other neutral lipids and phospholipids. In one case examined for gangliosides, these were increased twentyfold. One Gaucher spleen, in which others had reported that the stored "cerebroside" contained predominantly lactose as the saccharide moiety, has been examined in detail and it has been established that the stored material was, in fact, glucocerebroside, ceramide lactoside levels not being significantly elevated. In a further nine cases glucose was the major sugar detected in the splenic lipids.
采用柱色谱法（以纤维素、硅酸和弗罗里硅土为固定相）和薄层色谱法对正常脾脏和戈谢病脾脏的脂质组分进行分离和纯化。采用一种新的水解方法，随后进行纸色谱法，用于糖部分的鉴定。采用一种非水解的比色法，使用蒽酮和苔黑酚，分别测定糖脂中的葡萄糖和半乳糖。对该方法的局限性进行了研究。对两名对照受试者和三名戈谢病患者的脾脏进行了详细检查。在所有戈谢病脾脏中，主要特征是葡糖脑苷脂大量蓄积；中性神经酰胺低聚糖水平可能在正常范围内，其他中性脂质和磷脂也是如此。在对神经节苷脂进行检测的一个病例中，其含量增加了20倍。对一个戈谢病脾脏进行了详细检查，其他研究报告称该脾脏中储存的“脑苷脂”主要以乳糖作为糖部分，现已确定储存物质实际上是葡糖脑苷脂，神经酰胺乳糖苷水平没有显著升高。在另外九个病例中，葡萄糖是脾脏脂质中检测到的主要糖类。
