Ulrich-Bott B, Klem B, Kaiser R, Spranger J, Cantz M
Institut für Pathochemie und Allgemeine Neurochemie, Universität Heidelberg, BRD.
Enzyme. 1987;38(1-4):262-6. doi: 10.1159/000469214.
Organs obtained at autopsy from a patient with sialidosis were analyzed for 'bound' sialic acid and their ganglioside and neutral glycolipid patterns determined. The water-soluble bound sialic acid was increased between 10- and 17-fold in visceral organs, but only about 2-fold in the brain, when compared to normal controls. Lipid-bound sialic acid was increased up to 8-fold in visceral organs due to elevated amounts of gangliosides GM3, GD3 and probably GM4 and LM1, whereas the brain showed no deviation from controls. An alteration of the neutral glycolipid pattern was also observed. The results indicate an impaired catabolism of gangliosides in sialidosis in addition to that of sialyloligosaccharides and sialoglycoproteins.
对一名患唾液酸沉积症患者尸检时获取的器官进行分析，测定其“结合”唾液酸以及神经节苷脂和中性糖脂模式。与正常对照相比，水溶性结合唾液酸在内脏器官中增加了10至17倍，但在大脑中仅增加了约2倍。由于神经节苷脂GM3、GD3以及可能还有GM4和LM1的量增加，脂质结合唾液酸在内脏器官中增加了8倍，而大脑与对照无偏差。还观察到中性糖脂模式的改变。结果表明，除了唾液酸寡糖和唾液糖蛋白的分解代谢受损外，唾液酸沉积症中神经节苷脂的分解代谢也受损。
