Mersmann G, Von Figura K, Buddecke E
Hoppe Seylers Z Physiol Chem. 1976 May;357(5):641-8. doi: 10.1515/bchm2.1976.357.1.641.
Skin fibroblasts from healthy individuals and a mannosidosis patient were cultured in the presence of [2-3H] mannose and the cell homogenates were fractionated by trichloroacetic acid precipitation into a precipitable and a non-precipitable portion. In uptake as well as in chase experiments the precipitable fractions show no significant difference in their content of radioactivity, while an increased level of radioactivity is found in the non-precipitable fraction of mannosidosis cells. This higher radioactivity content is due to a higher mannose content and is caused by a slower degradation of this fraction. The differences between the metabolisms of the two cell lines can be expressed by the ratio of radioactivity in the non-precipitable and the precipitable fractions. This value is about three times higher for mannosidosis than for control cells. Pig kidney alpha-mannosidase is taken up by both cell lines and is able to correct the impaired degradation of the non-precipitable material in mannosidosis cells, as shown by the normalization of the above defined ratio of radioactivity for this cell type.
在[2-³H]甘露糖存在的情况下培养来自健康个体和一名甘露糖苷贮积症患者的皮肤成纤维细胞，然后用三氯乙酸沉淀法将细胞匀浆分离成可沉淀部分和不可沉淀部分。在摄取实验和追踪实验中，可沉淀部分的放射性含量均无显著差异，而在甘露糖苷贮积症细胞的不可沉淀部分中发现放射性水平有所升高。这种较高的放射性含量是由于甘露糖含量较高，并且是由该部分较慢的降解所致。两种细胞系代谢之间的差异可以通过不可沉淀部分与可沉淀部分的放射性比值来表示。对于甘露糖苷贮积症细胞，该值约为对照细胞的三倍。两种细胞系均摄取猪肾α-甘露糖苷酶，并且如通过该细胞类型上述定义的放射性比值的正常化所示，该酶能够纠正甘露糖苷贮积症细胞中不可沉淀物质降解受损的情况。
