Elleder M, Goebel H H, Koppang N
Hlava's Institute of Pathology, School of Medicine, Prague, Czechoslovakia.
Adv Exp Med Biol. 1989;266:243-58. doi: 10.1007/978-1-4899-5339-1_17.
Concanavalin A (Con A) binding to lipopigments (LPs) of the lipofuscin type was proved to be due to the high content of mannose. Two mannose bearing compounds could be recognized due to their different organic solvent solubility. One was best soluble in modified chloroform-methanol-water mixture (10:10:3) and corresponded most probably to the oligosaccharyl disphosphodolichol (oligo-PP-Dol) described to be significantly increased in LPs of inherited type. The second one, organic solvent insoluble corresponded to a glycoprotein (GP). The ratio of the two components varied. The deposition of the typical lipofuscin (age pigment) was dominated by the GP component. Its amount was greatest in neurolipofuscin (especially in the olivary nucleus) but very little in hepatocytic lipofuscin. In human neuronal ceroid lipofuscinoses (of early juvenile, and juvenile types) both components were found in large quantities in the storage granules of the affected neurons. The "protein type variant" of the storage material (Elleder, 1978) displayed the highest degree of lipid-bound mannose accumulation, the GP component being absent. In the late infantile, infantile and Kufs variants studied in paraffin sections only, the GP component was detectable, too as in the case of the secondary neuronal LP in mucopolysaccharidoses and gangliosidoses. In the canine model of NCL lipid bound mannose clearly predominated, the GP component being in low amount on average. Neither of the Con A reactive glycoconjugates could be identified as the component responsible for autofluorescence. However, both are most probably responsible for PAS positivity of lipofuscins. There were no detectable Con A reactive glycoconjugates in the histiocytic ceroid.
刀豆球蛋白A（Con A）与脂褐素类型的脂色素（LPs）结合被证明是由于甘露糖含量高。由于两种含甘露糖化合物在有机溶剂中的溶解度不同，可以将它们识别出来。一种在改良的氯仿 - 甲醇 - 水混合物（10:10:3）中溶解度最佳，最有可能对应于据描述在遗传性LPs中显著增加的寡糖基二磷酸多萜醇（oligo - PP - Dol）。第二种不溶于有机溶剂，对应于一种糖蛋白（GP）。这两种成分的比例各不相同。典型脂褐素（老年色素）的沉积以GP成分为主。其含量在神经脂褐素中最高（尤其是在橄榄核中），而在肝细胞脂褐素中很少。在人类早发性和青少年型神经元蜡样脂褐质沉积症中，在受影响神经元的储存颗粒中发现这两种成分都大量存在。储存物质的“蛋白类型变体”（埃勒德，1978年）显示出脂质结合甘露糖积累的最高程度，不存在GP成分。仅在石蜡切片中研究的晚婴儿型、婴儿型和库夫斯变体中，也可检测到GP成分，就像在黏多糖贮积症和神经节苷脂贮积症中的继发性神经元LP一样。在NCL的犬模型中，脂质结合甘露糖明显占主导，GP成分平均含量较低。Con A反应性糖结合物均未被鉴定为负责自发荧光的成分。然而，两者很可能是脂褐素PAS阳性的原因。在组织细胞蜡样质中未检测到Con A反应性糖结合物。
