Walkley S U, Wurzelmann S, Rattazzi M C, Baker H J
Department of Neuroscience, Rose F. Kennedy Center for Research in Mental Retardation and Human Development, Albert Einstein College of Medicine, Bronx, NY 10461.
Brain Res. 1990 Feb 26;510(1):63-73. doi: 10.1016/0006-8993(90)90728-t.
Golgi and combined Golgi-electron microscopic (EM) studies were carried out on cats in the terminal stages of GM2 ganglioside storage disease and the resulting data were compared with those from similar studies of other neuronal storage diseases in cats, including GM1 gangliosidosis. The results support the view that only limited types of neurons affected by the lysosomal hydrolase deficiency and subsequent intracellular storage have the capacity to sprout new dendritic-like growth processes from their axon hillocks, and that these neurons are essentially the same in all of these diseases studied to date. Golgi studies of CNS tissues from GM2 gangliosidosis cats revealed ectopic neurite growth on pyramidal neurons of cerebral cortex and multipolar cells of amygdala and claustrum, whereas other types of neurons responded to the metabolic defect with aspiny meganeurite formation or somatic enlargement, or appeared normal in terms of soma-dendritic morphology. Combined Golgi-EM studies of cortical pyramidal neurons revealed that ectopic, axon hillock neurites commonly possessed asymmetrical synapses which were similar to those observed in other storage disorders.
对患有GM2神经节苷脂贮积病终末期的猫进行了高尔基染色和高尔基染色与电子显微镜（EM）联合研究，并将所得数据与对猫的其他神经元贮积病（包括GM1神经节苷脂病）的类似研究数据进行了比较。结果支持这样一种观点，即只有受溶酶体水解酶缺乏及随后细胞内贮积影响的有限类型神经元才有能力从其轴丘萌发出新的树突状生长过程，而且在迄今为止研究的所有这些疾病中，这些神经元基本相同。对患有GM2神经节苷脂病的猫的中枢神经系统组织进行的高尔基染色研究显示，大脑皮质锥体神经元以及杏仁核和屏状核的多极细胞上有异位神经突生长，而其他类型的神经元对代谢缺陷的反应是形成无棘巨神经突或胞体增大，或者在胞体-树突形态方面看起来正常。对皮质锥体神经元进行的高尔基染色与电子显微镜联合研究显示，异位的轴丘神经突通常具有不对称突触，这与在其他贮积病中观察到的突触相似。
