Hardy B, Teitelman-Weissman B, Chazan S, Neri A
Biomed Pharmacother. 1987;41(1):40-4.
Glucocerebroside in Gaucher disease is stored in large Gaucher cells of monocyte-macrophage origin. Our aim was to achieve storage of this substrate in monocytes in vitro, in order to mimick the Gaucher cells in vivo. The method described using glucocerebroside-albumin complex, enabled such uptake and storage. The monocytes used changed to large hellical cells. Glucocerebrosidase activity was not affected by the accumulation and storage of the substrate. Although morphological evidence for the accumulation of glucocerebroside within the lysosomes has not yet been obtained, we suggest that these monocytes might represent an in vitro model for the study of Gaucher cells.
戈谢病中的葡萄糖脑苷脂储存在源自单核细胞-巨噬细胞的大型戈谢细胞中。我们的目的是在体外使这种底物储存在单核细胞中,以模拟体内的戈谢细胞。所描述的使用葡萄糖脑苷脂-白蛋白复合物的方法能够实现这种摄取和储存。所用的单核细胞变成了大型螺旋状细胞。葡萄糖脑苷脂酶活性不受底物积累和储存的影响。虽然尚未获得葡萄糖脑苷脂在溶酶体内积累的形态学证据,但我们认为这些单核细胞可能代表了用于研究戈谢细胞的体外模型。