Alroy J, Goyal V, Warren C D
Department of Pathology, Tufts University School of Medicine, Boston, MA 02111.
Acta Neuropathol. 1988;76(2):109-14. doi: 10.1007/BF00688094.
Lectin histochemical studies were performed on paraffin-embedded brain, spinal cord and eye sections of 16 animals from four different species affected with GM1- and GM2-gangliosidosis to identify specific carbohydrate residues in the perikaryon of neurons. We examined tissues from cats, cattle and dogs with GM1-gangliosidosis and from cats, dogs, and swine with GM2-gangliosidosis and compared them to corresponding normal animals. In all but two cases, the neurons stained intensely with Concanavalia ensiformis agglutinin (Con A); in 12 cases they stained with Dolichos biflorus agglutinin; in 10 cases with Ulex europaeus agglutinin-I; in 9 cases with Griffonia simplicifolia-I; and 8 with soybean agglutinin. Neurons from control tissues stained weakly with Con A, but not with any of the other lectins used. Similar staining patterns of neurons were noted in animals affected with the same disorder originating from the same mutant line. These findings highlight the fact that in gangliosidosis, the lectin staining patterns of neurons may be influenced by the deficiency in enzyme activity and by additional unknown but inherited factors.
对来自四个不同物种的16只患有GM1和GM2神经节苷脂贮积症的动物的石蜡包埋脑、脊髓和眼切片进行凝集素组织化学研究,以鉴定神经元胞体中的特定碳水化合物残基。我们检查了患有GM1神经节苷脂贮积症的猫、牛和狗以及患有GM2神经节苷脂贮积症的猫、狗和猪的组织,并将它们与相应的正常动物进行比较。除两例外,所有病例中的神经元均被刀豆球蛋白A(Con A)强烈染色;12例被双花扁豆凝集素染色;10例被荆豆凝集素-I染色;9例被西非单叶豆凝集素-I染色;8例被大豆凝集素染色。对照组织中的神经元被Con A弱染色,但未被所用的任何其他凝集素染色。在来自同一突变系的患有相同疾病的动物中,观察到神经元的类似染色模式。这些发现突出了这样一个事实,即在神经节苷脂贮积症中,神经元的凝集素染色模式可能受酶活性缺乏以及其他未知但可遗传因素的影响。