Philippart M, Sarlieve L, Meurant C, Mechler L
J Lipid Res. 1971 Jul;12(4):434-41.
The excretion of sulfatides in human urine was studied. 24-hr urine collections were filtered. Urinary glycolipids were extracted from the filter paper and fractionated on diethylaminoethyl cellulose and silicic acid columns, and by thin-layer chromatography. Fatty acids and long-chain bases were analyzed by gas-liquid chromatography of the corresponding esters and aldehydes. Glycosyl ceramide concentration was determined by gas-liquid chromatography of the trimethylsilyl ethers of the methyl glycosides. Normal females were found to excrete larger amounts of dihexosyl ceramides than males. Sulfatides were detected in all urine specimens. In sulfatidosis, a hereditary sulfatide storage disorder known as metachromatic leukcdystrophy, a large increase in sulfatide was readily apparent on a thin-layer chromatogram of the crude lipid extract. On comparing samples from normal individuals and patients with sulfatidosis, urinary sulfatide composition was remarkably similar to that previously reported in the kidney, including differences in fatty acid pattern. The determination of urinary sulfatides was a valuable confirmation of the deficiency in arylsulfatase A activity characteristic of sulfatidosis.
对人尿中硫脂的排泄情况进行了研究。收集24小时尿液并进行过滤。从滤纸上提取尿糖脂，并在二乙氨基乙基纤维素柱和硅酸柱上进行分级分离，同时采用薄层色谱法。通过对相应酯和醛的气液色谱分析来测定脂肪酸和长链碱。通过对甲基糖苷的三甲基硅醚进行气液色谱分析来测定糖基神经酰胺的浓度。结果发现，正常女性排泄的二己糖神经酰胺量比男性多。在所有尿液标本中均检测到了硫脂。在硫脂贮积症（一种称为异染性脑白质营养不良的遗传性硫脂贮积障碍）中，在粗脂质提取物的薄层色谱图上很容易看出硫脂大量增加。比较正常人和硫脂贮积症患者的样本时，尿硫脂组成与先前在肾脏中报道的情况非常相似，包括脂肪酸模式的差异。尿硫脂的测定是对硫脂贮积症所特有的芳基硫酸酯酶A活性缺乏的一项有价值的验证。
