Bianco P, Bonucci E
Basic Appl Histochem. 1986;30(4):409-16.
Plastic-embedded bone marrow biopsies from four patients with Gaucher's disease have been studied histochemically. Concanavalin A (ConA) was found to bind to cytoplasmic inclusions of Gaucher cells; the binding was prevented by lipid extraction or beta-glucosidase digestion. This suggests that glucocerebrosides stored in Gaucher cells are responsible for ConA binding; ConA staining combined with lipid extraction and beta-glucosidase digestion tests may be taken as a tool for the demonstration of Gaucher's cerebrosides of possible practical importance in diagnosis and investigation of Gaucher's disease. An excess of vic-glycol groups with respect to ConA binding-sugar residues and not extractable by lipid solvents are demonstrable in Gaucher cells. Vic-glycols appear to be regularly arranged at the electron microscopy level within Gaucher cell lysosomes along typical Gaucher "tubules", where some kind of interaction between lipid and protein should occur. Acid phosphatase might be one protein species involved in such interaction.
对4例戈谢病患者的塑料包埋骨髓活检组织进行了组织化学研究。发现伴刀豆球蛋白A(ConA)可与戈谢细胞的胞质内含物结合;脂质提取或β-葡萄糖苷酶消化可阻止这种结合。这表明戈谢细胞中储存的葡萄糖脑苷脂是ConA结合的原因;ConA染色结合脂质提取和β-葡萄糖苷酶消化试验可作为一种工具,用于证明在戈谢病诊断和研究中可能具有实际重要性的戈谢脑苷脂。在戈谢细胞中可证明相对于ConA结合糖残基存在过量的邻二醇基团,且不能被脂质溶剂提取。在电子显微镜水平上,邻二醇似乎沿着典型的戈谢“小管”在戈谢细胞溶酶体内规则排列,在那里脂质和蛋白质之间应该会发生某种相互作用。酸性磷酸酶可能是参与这种相互作用的一种蛋白质。