Yatziv S, Newburg D S, Livni N, Barfi G, Kolodny E H
Department of Pediatrics, Hadassah University Hospital Medical School, Jerusalem.
J Lab Clin Med. 1988 Apr;111(4):416-20.
Human blood-derived macrophages were cultured in the presence of conduritol-B-epoxide, a specific inhibitor of beta-glucosidase, to induce changes resembling those occurring in the cells of patients with Gaucher's disease. After 24 hours of incubation, only 5% of the original beta-glucosidase activity remained; on removal of the inhibitor, the enzyme activity recovered almost fully to control levels after 5 days. After 30 days of incubation with conduritol-B-epoxide, the macrophages contained almost 10 times as much glucocerebroside as the untreated controls, and the cells displayed morphologic changes reminiscent of Gaucher's cells. This in vitro system may enable detailed studies on the pathogenetic mechanisms associated with glucocerebroside accumulation in human macrophages as well as on the turnover of the accumulated substrate and reversal of the morphologic abnormalities on removal of the inhibitor.
将人血源性巨噬细胞在β-葡萄糖苷酶的特异性抑制剂环氧扁枝衣醇存在的情况下进行培养，以诱导出类似于戈谢病患者细胞中发生的变化。孵育24小时后，仅保留了原始β-葡萄糖苷酶活性的5%；去除抑制剂后，酶活性在5天后几乎完全恢复到对照水平。在用环氧扁枝衣醇孵育30天后，巨噬细胞所含的葡糖脑苷脂几乎是未处理对照的10倍，并且细胞呈现出类似于戈谢细胞的形态学变化。这个体外系统可能有助于对与人类巨噬细胞中葡糖脑苷脂积累相关的发病机制、积累底物的周转以及去除抑制剂后形态学异常的逆转进行详细研究。
