成人戈谢病患者白细胞中乳糖基神经酰胺的蓄积。

Accumulation of lactosyl ceramide in leukocytes of patients with adult Gaucher's disease.

作者信息

Klibansky C, Ossimi Z, Matoth Y, Pinkhas J, de Vries A

出版信息

Clin Chim Acta. 1976 Oct 1;72(1):141-6. doi: 10.1016/0009-8981(76)90045-0.

Abstract

Glycosphingolipids were isolated from the leukocytes of 10 patients with Type I, chronic nonneuronopathic (adult) Gaucher's disease and 12 normal subjects, by silicic acid column chromatography and thin-layer chromatography. Quantitation of the individual glycosyl ceramides was achieved by the determination of hexose and sphingosine content, using colorimetric and fluorometric procedures. Lactosyl ceramide, which is the main glycolipid in leukocytes of normal subjects, was significantly increased in the leukocytes of patients with Gaucher's disease. On the other hand, the amount of glycosyl ceramide, which is the main glycolipid accumulating in the reticuloendothelial cells of patients with Gaucher's disease, was similar in Gaucher and in control leukocytes.

摘要

通过硅酸柱色谱法和薄层色谱法，从10例I型慢性非神经元性（成人）戈谢病患者的白细胞以及12名正常受试者的白细胞中分离出糖鞘脂。采用比色法和荧光法，通过测定己糖和鞘氨醇含量对各糖基神经酰胺进行定量。乳糖基神经酰胺是正常受试者白细胞中的主要糖脂，在戈谢病患者的白细胞中显著增加。另一方面，戈谢病患者网状内皮细胞中积累的主要糖脂糖基神经酰胺的量，在戈谢病患者和对照白细胞中相似。